Cutis is a peer-reviewed clinical journal for the dermatologist, allergist, and general practitioner published monthly since 1965. Concise clinical articles present the practical side of dermatology, helping physicians to improve patient care. Cutis is referenced in Index Medicus/MEDLINE and is written and edited by industry leaders.
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Identifying safe, effective, and affordable evidence-based dermatologic treatments for older adults can be challenging because of age-related changes in the skin, comorbidities, polypharmacy, mobility issues, and cognitive changes. Phototherapy has been shown to be an effective nonpharmacologic treatment option for multiple challenging dermatologic conditions1-8; however, few studies have specifically examined its effectiveness in older adults. The challenge for older patients with psoriasis and dermatitis is that the conditions can be difficult to control and often require multiple treatment modalities.9,10 Patients with psoriasis also have a higher risk for diabetes, dyslipidemia, and cardiovascular disease compared to other older patients,11,12 which poses treatment challenges and makes nonpharmacologic treatments even more appealing.
Recent studies show that phototherapy can help decrease the use of dermatologic medications. Foerster and colleagues2 found that adults with psoriasis who were treated with phototherapy significantly decreased their use of topical steroids (24.5% fewer patients required steroid creams and 31.1% fewer patients required psoriasis-specific topicals)(P<.01) while their use of non–psoriasis-specific medications did not change. Click and colleagues13 identified a decrease in medication costs, health care utilization, and risk for immunosuppression in patients treated with phototherapy when compared to those treated with biologics and apremilast. Methotrexate is a common dermatologic medication that is highly associated with increased risks in elderly patients because of impaired immune system function and the presence of comorbidities (eg, kidney disease, obesity, diabetes, fatty liver),14 which increase in prevalence with age. Combining phototherapy with methotrexate can substantially decrease the amount of methotrexate needed to achieve disease control,15thereby decreasing the methotrexate-associated risks. Findings from these studies suggest that a safe, effective, cost-effective, and well-tolerated nonpharmacologic alternative, such as phototherapy, is highly desirable and should be optimized. Unfortunately, most studies that report the effectiveness of phototherapy are in younger populations.
This retrospective study aimed to (1) identify the most common dermatologic conditions treated with phototherapy in older adults, (2) examine the effectiveness and safety of phototherapy in older adults, and (3) compare the outcomes with 2 similar studies in the United Kingdom16 and Turkey.17
Methods
Design, Setting, Sample, and Statistical Analysis The institutional review boards of Kaiser Permanente Washington Health Research Institute, Seattle, and the University of Washington, Seattle, approved this study. It was conducted in a large US multispecialty health care system (Group Health, Seattle, Washington [now Kaiser Permanente Washington]) serving approximately 600,000 patients, using billing records to identify all patients treated with phototherapy between January 1, 2015, and December 31, 2015, all who received narrowband UVB (NB-UVB) phototherapy. All adults 65 years and older who received phototherapy treatment during the 12-month study period were included. Patients were included regardless of comorbidities and other dermatologic treatments to maintain as much uniformity as possible between the present study and 2 prior studies examining phototherapy in older adult populations in the United Kingdom16 and Turkey.17 Demographic and clinical factors were presented using frequencies (percentages) or means and medians as appropriate. Comparisons of dermatologic conditions and clearance levels used a Fisher exact test. The number of phototherapy treatments to clearance and total number of treatments were compared between groups of patients using independent sample t tests.
Phototherapy Protocol All patients received treatments administered by specially trained phototherapy nurses using a Daavlin UV Series (The Daavlin Company) or an Ultralite unit (Ultralite Enterprises, Inc), both with 48 lamps. All phototherapy nurses had been previously trained to provide treatments based on standardized protocols (Table 1) and to determine the patient’s level of disease clearance using a high to low clearance scale (Table 2). Daavlin’s treatment protocols were built into the software that accompanied the units and were developed based on the American Academy of Dermatology guidelines. The starting dose for an individual patient was determined based on the estimated minimal erythema dose for each phototype. If the patient was using photosensitizing medications, then the protocol guided the nurse to start the patient at a lower dose appropriate for their phototype. Patients with vitiligo were treated with the same starting and escalation doses as patients with Fitzpatrick phototype I because of the assumption that their vitiliginous skin had an increased risk for photosensitivity. A more recent review of the evidence has indicated that this assumption was overly conservative,18 and Kaiser Permanente Washington’s vitiligo protocol has been adjusted.
Results
Patients Billing records identified 229 total patients who received phototherapy in 2015, of whom 52 (22.7%) were at least 65 years old. The median age was 70 years (range, 65–91 years). Twenty-nine (56%) were men and 35 (67%) had previously received phototherapy treatments.
Dermatologic Conditions Treated With Phototherapy Our primary aim was to identify the most common dermatologic conditions treated with phototherapy in older adults. Psoriasis and dermatitis were the most common conditions treated in the sample (50% [26/52] and 21% [11/52], respectively), with mycosis fungoides being the third most common (10% [5/52]) and vitiligo tied with prurigo nodularis as fourth most common (6% [3/52])(Figure 1).
Figure 1. Dermatologic conditions of older patients (N=52). Percentages were rounded to the nearest whole number.
Effectiveness and Safety of Phototherapy Our secondary aim was to examine the effectiveness and safety of phototherapy in older adults. Phototherapy was effective in this population, with 50 of 52 patients (96%) achieving a high or medium level of clearance. The degree of clearance for each of the dermatologic conditions is shown in Figure 2. Psoriasis and dermatitis achieved high clearance rates in 81% (21/26) and 82% (9/11) of patients, respectively. Overall, conditions did not have significant differences in clearances rates (Fisher exact test, P=.10). On average, it took patients 33 treatments to achieve medium or high rates of clearance. Psoriasis cleared more quickly, with an average of 30.4 treatments vs 36.1 treatments for other conditions, but the difference was not significant (t test, P=.26). Patients received an average of 98 total phototherapy treatments; the median number of treatments was 81 due to many being on maintenance therapy over several months. There was no relationship between a history of treatment with phototherapy and the total number of treatments needed to achieve clearance (t test, P=.40), but interestingly, those who had a history of phototherapy took approximately 5 more treatments to achieve clearance. The present study found that a slightly larger number of men were being treated for psoriasis (15 men vs 11 women), but there was no significant difference in response rate based on gender.
Figure 2. Degree of clearance by dermatologic condition.
Side effects from phototherapy were minimal; 24 patients (46%) experienced grade 1 (mild) erythema at some point during their treatment course. Thirteen (25%) patients experienced grade 2 erythema, but this was a rare event for most patients. Only 1 (2%) patient experienced grade 3 erythema 1 time. Three patients experienced increased itching (6%). Thirteen (25%) patients had no side effects. None developed severe erythema or blisters, and none discontinued phototherapy because of side effects. Over the course of the study year, we found a high degree of acceptance of phototherapy treatments by older patients: 22 (42%) completed therapy after achieving clearance, 10 (19%) were continuing ongoing treatments (maintenance), and 15 (29%) stopped because of life circumstances (eg, other health issues, moving out of the area). Only 4 (8%) stopped because of a lack of effectiveness, and 1 (2%) patient because the treatments were burdensome.
Comparison of Outcomes Our third aim was to compare the outcomes with similar studies in the United Kingdom16 and Turkey.17 This study confirmed that phototherapy is being used in older adults (22.7% of this study’s total patients) and is an effective treatment for older patients experiencing a range of challenging inflammatory and proliferative skin diseases similar to studies in the general population. Prior phototherapy studies in elderly patients also found psoriasis to be the most common skin condition treated, with 1 study finding that 51% (19/37) of older phototherapy patients had psoriasis,16 while another reported 58% (37/95) of older phototherapy patients had psoriasis.17 These numbers are similar to those in our study, which showed 50% (26/52) of elderly phototherapy patients had psoriasis. Psoriasis is the main indication for treatment with NB-UVB phototherapy in the general population,19 and because the risk for psoriasis increases with age,20 it is not surprising that all 3 studies found psoriasis to be the most common indication in elderly phototherapy patients. Table 3 provides further details on conditions treated in all 3 studies.
Comment
Our study found that 94% of patients with psoriasis achieved clearance with an average of 30.4 treatments, which is comparable to the reported 91% response rate with an average of 30 treatments in the United Kingdom.16 The other similar study in Turkey17 reported 73.7% of psoriasis patients achieved a 75% or more improvement from baseline with an average of 42 treatments, which may reflect underlying differences in regional skin type. Of note, the scatter chart (Figure 3) shows that several patients in the present study’s analysis are listed as not clear, but many of those patients had low treatment numbers below the mean time to clearance. Thus, the present study’s response rate may have been underestimated.
Figure 3. Comparison of total treatments and side effects across all conditions. MF indicates mycosis fungoides; DNC, did not clear. Bold rule indicates patients who experienced side effects greater than grade 1.
In the general population, studies show that psoriasis treated with standardized phototherapy protocols typically clears with an average of 20.6 treatments.21 The levels of clearance were similar in our study’s older population, but more treatments were required to achieve those results, with an average of 10 more treatments needed (an additional 3.3 weeks). Similar results were found in this sample for dermatitis and mycosis fungoides, indicating comparable clearance rates and levels but a need for more treatments to achieve similar results compared to the general population.
Additionally, in the current study more patients experienced grade 1 (mild) erythema (46%) and grade 2 erythema (25%) at some point in their treatment compared with the United Kingdom16 (1.89%) and Turkey17 (35%) studies, though these side effects did not impact the clearance rate. Interestingly, the current study’s scatter chart (Figure 3) illustrates that this side effect did not seem to increase with aging in this population. If anything, the erythema response was more prevalent in the median or younger patients in the sample. Erythema may have been due to the frequent use of photosensitizing medications in older adults in the United States, some of which typically get discontinued in patients 75 years and older (eg, statins). Other potential causes might include the use of phototype vs minimal erythema dose–driven protocols, the standard utilization of protocols originally designed for psoriasis vs other condition-specific protocols, missed treatments leading to increased sensitivity, or possibly shielding mishaps (eg, not wearing a prescribed face shield). Given the number of potential causes and the possibility of overlapping factors, careful analysis is important. With NB-UVB phototherapy, near-erythemogenic doses are optimal to achieve effective treatments, but this delicate balance may be more problematic for older adults. Future studies are needed to fully determine the factors at play for this population. In the interim, it is important for phototherapy-trained nurses to consider this risk carefully in the older population. They must follow the prescribed protocols that guide them to query patients about their responses to the prior treatment (eg, erythema, tenderness, itching), photosensitizing medications, missed treatments, and placement of shielding, and then adjust the treatment dosing accordingly.
Limitations This study had several limitations. Although clinical outcomes were recorded prospectively, the analysis was retrospective, unblinded, and not placebo controlled. It was conducted in a single organization (Group Health [now Kaiser Permanente Washington]) but did analyze data from 4 medical centers in different cities with diverse demographics and a variety of nursing staff providing the treatments. Although the vitiligo treatment protocol likely slowed the response rate for those patients with vitiligo, the numbers were small (ie, only 3 of 52 patients), so the researchers chose to include them in the current study. The sample population was relatively small, but when these data are evaluated alongside the studies in the United Kingdom16 and Turkey,17 they show a consistent picture illustrating the effectiveness and safety of phototherapy in the older population. Further epidemiologic studies could be helpful to further describe the usefulness of this modality compared with other treatments for a variety of dermatoses in this age group. Supplementary analysis specifically examining the relationship between the number and type of photosensitizing medications, frequency of erythema, and time to clearance also could be useful.
Conclusion
Older adults with a variety of dermatoses respond well to phototherapy and should have the opportunity to use it, particularly considering the potential for increased complications and costs from other treatment modalities, such as commonly used immunosuppressive pharmaceuticals. However, the current study and the comparison studies indicate that it is important to carefully consider the slower clearance rates and the potential risk for increased erythema in this population and adjust patient education and treatment dosing accordingly.
Unfortunately, many dermatology centers do not offer phototherapy because of infrastructure limitations such as space and specially trained nursing staff. Increasing accessibility of phototherapy for older adults through home treatments may be an alternative, given its effectiveness in the general population.22,23 In addition, home phototherapy may be worth pursuing for the older population considering the challenges they may face with transportation to the clinic setting and their increased risk for serious illness if exposed to infections such as COVID-19. The COVID-19 pandemic has brought to light the need for reliable, safe, and effective treatments that can be utilized in the safety of patients’ homes and should therefore be considered as an option for older adults. Issues such as mobility and cognitive decline could pose some complicating factors, but with the help of a well-trained family member or caregiver, home phototherapy could be a viable option that improves accessibility for older patients. Future research opportunities include further examination of the slower but ultimately equivalent response to phototherapy in the older population, the influence of photosensitizing medications on phototherapy effects, and the impact of phototherapy on utilization of immunosuppressive pharmaceuticals in older adults.
References
British Photodermatology Group. An appraisal of narrowband (TL-01) UVB phototherapy. British Photodermatology Group Workshop Report (April 1996). Br J Dermatol. 1997;137:327-330.
Foerster J, Boswell K, West J, et al. Narrowband UVB treatment is highly effective and causes a strong reduction in the use of steroid and other creams in psoriasis patients in clinical practice. PLoS ONE. 2017;12:e0181813. doi:10.1371/journal.pone.0181813
Fernández-Guarino M, Aboin-Gonzalez S, Barchino L, et al. Treatment of moderate and severe adult chronic atopic dermatitis with narrow-band UVB and the combination of narrow-band UVB/UVA phototherapy. Dermatol Ther. 2015;29:19-23.
Ryu HH, Choe YS, Jo S, et al. Remission period in psoriasis after multiple cycles of narrowband ultraviolet B phototherapy. J Dermatol. 2014;41:622-627.
Tintle S, Shemer A, Suárez-Fariñas M, et al. Reversal of atopic dermatitis with narrow-band UVB phototherapy and biomarkers for therapeutic response. J Allergy Clin Immunol. 2011;128:583-593.
Gambichler T, Breuckmann F, Boms S, et al. Narrowband UVB phototherapy in skin conditions beyond psoriasis. J Am Acad Dermatol. 2005;52:660-670.
Schneider LA, Hinrichs R, Scharffetter-Kochanek K. Phototherapy and photochemotherapy. Clin Dermatol. 2008;26:464-476.
Martin JA, Laube S, Edwards C, et al. Rate of acute adverse events for narrow-band UVB and psoralen-UVA phototherapy. Photodermatol Photoimmunol Photomed. 2007;23:68-72.
Mokos ZB, Jovic A, Ceovic R, et al. Therapeutic challenges in the mature patient. Clin Dermatol. 2018;36:128-139.
Di Lernia V, Goldust M. An overview of the efficacy and safety of systemic treatments for psoriasis in the elderly. Exp Opin Biol Ther. 2018;18:897-903.
Napolitano M, Balato N, Ayala F, et al. Psoriasis in elderly and non-elderly population: clinical and molecular features. G Ital Dermatol Venereol. 2016;151:587-595.
Grozdev IS, Van Voorhees AS, Gottlieb AB, et al. Psoriasis in the elderly: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol. 2011;65:537-545.
Click J, Alabaster A, Postlethwaite D, et al. Effect of availability of at-home phototherapy on the use of systemic medications for psoriasis. Photodermatol Photoimmunol Photomed. 2017;33:345-346.
Piaserico S, Conti A, Lo Console F, et al. Efficacy and safety of systemic treatments for psoriasis in elderly. Acta Derm Venereol. 2014;94:293-297.
Soliman A, Nofal E, Nofal A, et al. Combination therapy of methotrexate plus NB-UVB phototherapy is more effective than methotrexate monotherapy in the treatment of chronic plaque psoriasis. J Dermatol Treat. 2015;26:528-534.
Powell JB, Gach JE. Phototherapy in the elderly. Clin Exp Dermatol. 2015;40:605-610.
Bulur I, Erdogan HK, Aksu AE, et al. The efficacy and safety of phototherapy in geriatric patients: a retrospective study. An Bras Dermatol. 2018;93:33-38.
Madigan LM, Al-Jamal M, Hamzavi I. Exploring the gaps in the evidence-based application of narrowband UVB for the treatment of vitiligo. Photodermatol Photoimmunol Photomed. 2016;32:66-80.
Ibbotson SH. A perspective on the use of NB-UVB phototherapy vs. PUVA photochemotherapy. Front Med (Lausanne). 2018;5:184.
Bell LM, Sedlack R, Beard CM, et al. Incidence of psoriasis in Rochester, Minn, 1980-1983. Arch Dermatol. 1991;127:1184-1187.
Cameron H, Yule S, Dawe RS, et al. Review of an established UK home phototherapy service 1998-2011: improving access to a cost-effective treatment for chronic skin disease. Public Health. 2014;128:317-324.
Matthews SW, Simmer M, Williams L, et al. Transition of patients with psoriasis from office-based phototherapy to nurse-supported home phototherapy: a pilot study. JDNA. 2018;10:29-41.
From the University of Washington, Seattle. Drs. Matthews and Pike are from the School of Nursing. Dr. Chien is from the School of Medicine. Drs. Matthews and Chien also are from Kaiser Permanente Dermatology, Bellevue, Washington.
The authors report no conflict of interest.
Correspondence: Sarah W. Matthews, DNP, University of Washington, 1959 NE Pacific St, Box 357263, Seattle, WA 98195-7263 (sarahm09@uw.edu).
From the University of Washington, Seattle. Drs. Matthews and Pike are from the School of Nursing. Dr. Chien is from the School of Medicine. Drs. Matthews and Chien also are from Kaiser Permanente Dermatology, Bellevue, Washington.
The authors report no conflict of interest.
Correspondence: Sarah W. Matthews, DNP, University of Washington, 1959 NE Pacific St, Box 357263, Seattle, WA 98195-7263 (sarahm09@uw.edu).
Author and Disclosure Information
From the University of Washington, Seattle. Drs. Matthews and Pike are from the School of Nursing. Dr. Chien is from the School of Medicine. Drs. Matthews and Chien also are from Kaiser Permanente Dermatology, Bellevue, Washington.
The authors report no conflict of interest.
Correspondence: Sarah W. Matthews, DNP, University of Washington, 1959 NE Pacific St, Box 357263, Seattle, WA 98195-7263 (sarahm09@uw.edu).
Identifying safe, effective, and affordable evidence-based dermatologic treatments for older adults can be challenging because of age-related changes in the skin, comorbidities, polypharmacy, mobility issues, and cognitive changes. Phototherapy has been shown to be an effective nonpharmacologic treatment option for multiple challenging dermatologic conditions1-8; however, few studies have specifically examined its effectiveness in older adults. The challenge for older patients with psoriasis and dermatitis is that the conditions can be difficult to control and often require multiple treatment modalities.9,10 Patients with psoriasis also have a higher risk for diabetes, dyslipidemia, and cardiovascular disease compared to other older patients,11,12 which poses treatment challenges and makes nonpharmacologic treatments even more appealing.
Recent studies show that phototherapy can help decrease the use of dermatologic medications. Foerster and colleagues2 found that adults with psoriasis who were treated with phototherapy significantly decreased their use of topical steroids (24.5% fewer patients required steroid creams and 31.1% fewer patients required psoriasis-specific topicals)(P<.01) while their use of non–psoriasis-specific medications did not change. Click and colleagues13 identified a decrease in medication costs, health care utilization, and risk for immunosuppression in patients treated with phototherapy when compared to those treated with biologics and apremilast. Methotrexate is a common dermatologic medication that is highly associated with increased risks in elderly patients because of impaired immune system function and the presence of comorbidities (eg, kidney disease, obesity, diabetes, fatty liver),14 which increase in prevalence with age. Combining phototherapy with methotrexate can substantially decrease the amount of methotrexate needed to achieve disease control,15thereby decreasing the methotrexate-associated risks. Findings from these studies suggest that a safe, effective, cost-effective, and well-tolerated nonpharmacologic alternative, such as phototherapy, is highly desirable and should be optimized. Unfortunately, most studies that report the effectiveness of phototherapy are in younger populations.
This retrospective study aimed to (1) identify the most common dermatologic conditions treated with phototherapy in older adults, (2) examine the effectiveness and safety of phototherapy in older adults, and (3) compare the outcomes with 2 similar studies in the United Kingdom16 and Turkey.17
Methods
Design, Setting, Sample, and Statistical Analysis The institutional review boards of Kaiser Permanente Washington Health Research Institute, Seattle, and the University of Washington, Seattle, approved this study. It was conducted in a large US multispecialty health care system (Group Health, Seattle, Washington [now Kaiser Permanente Washington]) serving approximately 600,000 patients, using billing records to identify all patients treated with phototherapy between January 1, 2015, and December 31, 2015, all who received narrowband UVB (NB-UVB) phototherapy. All adults 65 years and older who received phototherapy treatment during the 12-month study period were included. Patients were included regardless of comorbidities and other dermatologic treatments to maintain as much uniformity as possible between the present study and 2 prior studies examining phototherapy in older adult populations in the United Kingdom16 and Turkey.17 Demographic and clinical factors were presented using frequencies (percentages) or means and medians as appropriate. Comparisons of dermatologic conditions and clearance levels used a Fisher exact test. The number of phototherapy treatments to clearance and total number of treatments were compared between groups of patients using independent sample t tests.
Phototherapy Protocol All patients received treatments administered by specially trained phototherapy nurses using a Daavlin UV Series (The Daavlin Company) or an Ultralite unit (Ultralite Enterprises, Inc), both with 48 lamps. All phototherapy nurses had been previously trained to provide treatments based on standardized protocols (Table 1) and to determine the patient’s level of disease clearance using a high to low clearance scale (Table 2). Daavlin’s treatment protocols were built into the software that accompanied the units and were developed based on the American Academy of Dermatology guidelines. The starting dose for an individual patient was determined based on the estimated minimal erythema dose for each phototype. If the patient was using photosensitizing medications, then the protocol guided the nurse to start the patient at a lower dose appropriate for their phototype. Patients with vitiligo were treated with the same starting and escalation doses as patients with Fitzpatrick phototype I because of the assumption that their vitiliginous skin had an increased risk for photosensitivity. A more recent review of the evidence has indicated that this assumption was overly conservative,18 and Kaiser Permanente Washington’s vitiligo protocol has been adjusted.
Results
Patients Billing records identified 229 total patients who received phototherapy in 2015, of whom 52 (22.7%) were at least 65 years old. The median age was 70 years (range, 65–91 years). Twenty-nine (56%) were men and 35 (67%) had previously received phototherapy treatments.
Dermatologic Conditions Treated With Phototherapy Our primary aim was to identify the most common dermatologic conditions treated with phototherapy in older adults. Psoriasis and dermatitis were the most common conditions treated in the sample (50% [26/52] and 21% [11/52], respectively), with mycosis fungoides being the third most common (10% [5/52]) and vitiligo tied with prurigo nodularis as fourth most common (6% [3/52])(Figure 1).
Figure 1. Dermatologic conditions of older patients (N=52). Percentages were rounded to the nearest whole number.
Effectiveness and Safety of Phototherapy Our secondary aim was to examine the effectiveness and safety of phototherapy in older adults. Phototherapy was effective in this population, with 50 of 52 patients (96%) achieving a high or medium level of clearance. The degree of clearance for each of the dermatologic conditions is shown in Figure 2. Psoriasis and dermatitis achieved high clearance rates in 81% (21/26) and 82% (9/11) of patients, respectively. Overall, conditions did not have significant differences in clearances rates (Fisher exact test, P=.10). On average, it took patients 33 treatments to achieve medium or high rates of clearance. Psoriasis cleared more quickly, with an average of 30.4 treatments vs 36.1 treatments for other conditions, but the difference was not significant (t test, P=.26). Patients received an average of 98 total phototherapy treatments; the median number of treatments was 81 due to many being on maintenance therapy over several months. There was no relationship between a history of treatment with phototherapy and the total number of treatments needed to achieve clearance (t test, P=.40), but interestingly, those who had a history of phototherapy took approximately 5 more treatments to achieve clearance. The present study found that a slightly larger number of men were being treated for psoriasis (15 men vs 11 women), but there was no significant difference in response rate based on gender.
Figure 2. Degree of clearance by dermatologic condition.
Side effects from phototherapy were minimal; 24 patients (46%) experienced grade 1 (mild) erythema at some point during their treatment course. Thirteen (25%) patients experienced grade 2 erythema, but this was a rare event for most patients. Only 1 (2%) patient experienced grade 3 erythema 1 time. Three patients experienced increased itching (6%). Thirteen (25%) patients had no side effects. None developed severe erythema or blisters, and none discontinued phototherapy because of side effects. Over the course of the study year, we found a high degree of acceptance of phototherapy treatments by older patients: 22 (42%) completed therapy after achieving clearance, 10 (19%) were continuing ongoing treatments (maintenance), and 15 (29%) stopped because of life circumstances (eg, other health issues, moving out of the area). Only 4 (8%) stopped because of a lack of effectiveness, and 1 (2%) patient because the treatments were burdensome.
Comparison of Outcomes Our third aim was to compare the outcomes with similar studies in the United Kingdom16 and Turkey.17 This study confirmed that phototherapy is being used in older adults (22.7% of this study’s total patients) and is an effective treatment for older patients experiencing a range of challenging inflammatory and proliferative skin diseases similar to studies in the general population. Prior phototherapy studies in elderly patients also found psoriasis to be the most common skin condition treated, with 1 study finding that 51% (19/37) of older phototherapy patients had psoriasis,16 while another reported 58% (37/95) of older phototherapy patients had psoriasis.17 These numbers are similar to those in our study, which showed 50% (26/52) of elderly phototherapy patients had psoriasis. Psoriasis is the main indication for treatment with NB-UVB phototherapy in the general population,19 and because the risk for psoriasis increases with age,20 it is not surprising that all 3 studies found psoriasis to be the most common indication in elderly phototherapy patients. Table 3 provides further details on conditions treated in all 3 studies.
Comment
Our study found that 94% of patients with psoriasis achieved clearance with an average of 30.4 treatments, which is comparable to the reported 91% response rate with an average of 30 treatments in the United Kingdom.16 The other similar study in Turkey17 reported 73.7% of psoriasis patients achieved a 75% or more improvement from baseline with an average of 42 treatments, which may reflect underlying differences in regional skin type. Of note, the scatter chart (Figure 3) shows that several patients in the present study’s analysis are listed as not clear, but many of those patients had low treatment numbers below the mean time to clearance. Thus, the present study’s response rate may have been underestimated.
Figure 3. Comparison of total treatments and side effects across all conditions. MF indicates mycosis fungoides; DNC, did not clear. Bold rule indicates patients who experienced side effects greater than grade 1.
In the general population, studies show that psoriasis treated with standardized phototherapy protocols typically clears with an average of 20.6 treatments.21 The levels of clearance were similar in our study’s older population, but more treatments were required to achieve those results, with an average of 10 more treatments needed (an additional 3.3 weeks). Similar results were found in this sample for dermatitis and mycosis fungoides, indicating comparable clearance rates and levels but a need for more treatments to achieve similar results compared to the general population.
Additionally, in the current study more patients experienced grade 1 (mild) erythema (46%) and grade 2 erythema (25%) at some point in their treatment compared with the United Kingdom16 (1.89%) and Turkey17 (35%) studies, though these side effects did not impact the clearance rate. Interestingly, the current study’s scatter chart (Figure 3) illustrates that this side effect did not seem to increase with aging in this population. If anything, the erythema response was more prevalent in the median or younger patients in the sample. Erythema may have been due to the frequent use of photosensitizing medications in older adults in the United States, some of which typically get discontinued in patients 75 years and older (eg, statins). Other potential causes might include the use of phototype vs minimal erythema dose–driven protocols, the standard utilization of protocols originally designed for psoriasis vs other condition-specific protocols, missed treatments leading to increased sensitivity, or possibly shielding mishaps (eg, not wearing a prescribed face shield). Given the number of potential causes and the possibility of overlapping factors, careful analysis is important. With NB-UVB phototherapy, near-erythemogenic doses are optimal to achieve effective treatments, but this delicate balance may be more problematic for older adults. Future studies are needed to fully determine the factors at play for this population. In the interim, it is important for phototherapy-trained nurses to consider this risk carefully in the older population. They must follow the prescribed protocols that guide them to query patients about their responses to the prior treatment (eg, erythema, tenderness, itching), photosensitizing medications, missed treatments, and placement of shielding, and then adjust the treatment dosing accordingly.
Limitations This study had several limitations. Although clinical outcomes were recorded prospectively, the analysis was retrospective, unblinded, and not placebo controlled. It was conducted in a single organization (Group Health [now Kaiser Permanente Washington]) but did analyze data from 4 medical centers in different cities with diverse demographics and a variety of nursing staff providing the treatments. Although the vitiligo treatment protocol likely slowed the response rate for those patients with vitiligo, the numbers were small (ie, only 3 of 52 patients), so the researchers chose to include them in the current study. The sample population was relatively small, but when these data are evaluated alongside the studies in the United Kingdom16 and Turkey,17 they show a consistent picture illustrating the effectiveness and safety of phototherapy in the older population. Further epidemiologic studies could be helpful to further describe the usefulness of this modality compared with other treatments for a variety of dermatoses in this age group. Supplementary analysis specifically examining the relationship between the number and type of photosensitizing medications, frequency of erythema, and time to clearance also could be useful.
Conclusion
Older adults with a variety of dermatoses respond well to phototherapy and should have the opportunity to use it, particularly considering the potential for increased complications and costs from other treatment modalities, such as commonly used immunosuppressive pharmaceuticals. However, the current study and the comparison studies indicate that it is important to carefully consider the slower clearance rates and the potential risk for increased erythema in this population and adjust patient education and treatment dosing accordingly.
Unfortunately, many dermatology centers do not offer phototherapy because of infrastructure limitations such as space and specially trained nursing staff. Increasing accessibility of phototherapy for older adults through home treatments may be an alternative, given its effectiveness in the general population.22,23 In addition, home phototherapy may be worth pursuing for the older population considering the challenges they may face with transportation to the clinic setting and their increased risk for serious illness if exposed to infections such as COVID-19. The COVID-19 pandemic has brought to light the need for reliable, safe, and effective treatments that can be utilized in the safety of patients’ homes and should therefore be considered as an option for older adults. Issues such as mobility and cognitive decline could pose some complicating factors, but with the help of a well-trained family member or caregiver, home phototherapy could be a viable option that improves accessibility for older patients. Future research opportunities include further examination of the slower but ultimately equivalent response to phototherapy in the older population, the influence of photosensitizing medications on phototherapy effects, and the impact of phototherapy on utilization of immunosuppressive pharmaceuticals in older adults.
Identifying safe, effective, and affordable evidence-based dermatologic treatments for older adults can be challenging because of age-related changes in the skin, comorbidities, polypharmacy, mobility issues, and cognitive changes. Phototherapy has been shown to be an effective nonpharmacologic treatment option for multiple challenging dermatologic conditions1-8; however, few studies have specifically examined its effectiveness in older adults. The challenge for older patients with psoriasis and dermatitis is that the conditions can be difficult to control and often require multiple treatment modalities.9,10 Patients with psoriasis also have a higher risk for diabetes, dyslipidemia, and cardiovascular disease compared to other older patients,11,12 which poses treatment challenges and makes nonpharmacologic treatments even more appealing.
Recent studies show that phototherapy can help decrease the use of dermatologic medications. Foerster and colleagues2 found that adults with psoriasis who were treated with phototherapy significantly decreased their use of topical steroids (24.5% fewer patients required steroid creams and 31.1% fewer patients required psoriasis-specific topicals)(P<.01) while their use of non–psoriasis-specific medications did not change. Click and colleagues13 identified a decrease in medication costs, health care utilization, and risk for immunosuppression in patients treated with phototherapy when compared to those treated with biologics and apremilast. Methotrexate is a common dermatologic medication that is highly associated with increased risks in elderly patients because of impaired immune system function and the presence of comorbidities (eg, kidney disease, obesity, diabetes, fatty liver),14 which increase in prevalence with age. Combining phototherapy with methotrexate can substantially decrease the amount of methotrexate needed to achieve disease control,15thereby decreasing the methotrexate-associated risks. Findings from these studies suggest that a safe, effective, cost-effective, and well-tolerated nonpharmacologic alternative, such as phototherapy, is highly desirable and should be optimized. Unfortunately, most studies that report the effectiveness of phototherapy are in younger populations.
This retrospective study aimed to (1) identify the most common dermatologic conditions treated with phototherapy in older adults, (2) examine the effectiveness and safety of phototherapy in older adults, and (3) compare the outcomes with 2 similar studies in the United Kingdom16 and Turkey.17
Methods
Design, Setting, Sample, and Statistical Analysis The institutional review boards of Kaiser Permanente Washington Health Research Institute, Seattle, and the University of Washington, Seattle, approved this study. It was conducted in a large US multispecialty health care system (Group Health, Seattle, Washington [now Kaiser Permanente Washington]) serving approximately 600,000 patients, using billing records to identify all patients treated with phototherapy between January 1, 2015, and December 31, 2015, all who received narrowband UVB (NB-UVB) phototherapy. All adults 65 years and older who received phototherapy treatment during the 12-month study period were included. Patients were included regardless of comorbidities and other dermatologic treatments to maintain as much uniformity as possible between the present study and 2 prior studies examining phototherapy in older adult populations in the United Kingdom16 and Turkey.17 Demographic and clinical factors were presented using frequencies (percentages) or means and medians as appropriate. Comparisons of dermatologic conditions and clearance levels used a Fisher exact test. The number of phototherapy treatments to clearance and total number of treatments were compared between groups of patients using independent sample t tests.
Phototherapy Protocol All patients received treatments administered by specially trained phototherapy nurses using a Daavlin UV Series (The Daavlin Company) or an Ultralite unit (Ultralite Enterprises, Inc), both with 48 lamps. All phototherapy nurses had been previously trained to provide treatments based on standardized protocols (Table 1) and to determine the patient’s level of disease clearance using a high to low clearance scale (Table 2). Daavlin’s treatment protocols were built into the software that accompanied the units and were developed based on the American Academy of Dermatology guidelines. The starting dose for an individual patient was determined based on the estimated minimal erythema dose for each phototype. If the patient was using photosensitizing medications, then the protocol guided the nurse to start the patient at a lower dose appropriate for their phototype. Patients with vitiligo were treated with the same starting and escalation doses as patients with Fitzpatrick phototype I because of the assumption that their vitiliginous skin had an increased risk for photosensitivity. A more recent review of the evidence has indicated that this assumption was overly conservative,18 and Kaiser Permanente Washington’s vitiligo protocol has been adjusted.
Results
Patients Billing records identified 229 total patients who received phototherapy in 2015, of whom 52 (22.7%) were at least 65 years old. The median age was 70 years (range, 65–91 years). Twenty-nine (56%) were men and 35 (67%) had previously received phototherapy treatments.
Dermatologic Conditions Treated With Phototherapy Our primary aim was to identify the most common dermatologic conditions treated with phototherapy in older adults. Psoriasis and dermatitis were the most common conditions treated in the sample (50% [26/52] and 21% [11/52], respectively), with mycosis fungoides being the third most common (10% [5/52]) and vitiligo tied with prurigo nodularis as fourth most common (6% [3/52])(Figure 1).
Figure 1. Dermatologic conditions of older patients (N=52). Percentages were rounded to the nearest whole number.
Effectiveness and Safety of Phototherapy Our secondary aim was to examine the effectiveness and safety of phototherapy in older adults. Phototherapy was effective in this population, with 50 of 52 patients (96%) achieving a high or medium level of clearance. The degree of clearance for each of the dermatologic conditions is shown in Figure 2. Psoriasis and dermatitis achieved high clearance rates in 81% (21/26) and 82% (9/11) of patients, respectively. Overall, conditions did not have significant differences in clearances rates (Fisher exact test, P=.10). On average, it took patients 33 treatments to achieve medium or high rates of clearance. Psoriasis cleared more quickly, with an average of 30.4 treatments vs 36.1 treatments for other conditions, but the difference was not significant (t test, P=.26). Patients received an average of 98 total phototherapy treatments; the median number of treatments was 81 due to many being on maintenance therapy over several months. There was no relationship between a history of treatment with phototherapy and the total number of treatments needed to achieve clearance (t test, P=.40), but interestingly, those who had a history of phototherapy took approximately 5 more treatments to achieve clearance. The present study found that a slightly larger number of men were being treated for psoriasis (15 men vs 11 women), but there was no significant difference in response rate based on gender.
Figure 2. Degree of clearance by dermatologic condition.
Side effects from phototherapy were minimal; 24 patients (46%) experienced grade 1 (mild) erythema at some point during their treatment course. Thirteen (25%) patients experienced grade 2 erythema, but this was a rare event for most patients. Only 1 (2%) patient experienced grade 3 erythema 1 time. Three patients experienced increased itching (6%). Thirteen (25%) patients had no side effects. None developed severe erythema or blisters, and none discontinued phototherapy because of side effects. Over the course of the study year, we found a high degree of acceptance of phototherapy treatments by older patients: 22 (42%) completed therapy after achieving clearance, 10 (19%) were continuing ongoing treatments (maintenance), and 15 (29%) stopped because of life circumstances (eg, other health issues, moving out of the area). Only 4 (8%) stopped because of a lack of effectiveness, and 1 (2%) patient because the treatments were burdensome.
Comparison of Outcomes Our third aim was to compare the outcomes with similar studies in the United Kingdom16 and Turkey.17 This study confirmed that phototherapy is being used in older adults (22.7% of this study’s total patients) and is an effective treatment for older patients experiencing a range of challenging inflammatory and proliferative skin diseases similar to studies in the general population. Prior phototherapy studies in elderly patients also found psoriasis to be the most common skin condition treated, with 1 study finding that 51% (19/37) of older phototherapy patients had psoriasis,16 while another reported 58% (37/95) of older phototherapy patients had psoriasis.17 These numbers are similar to those in our study, which showed 50% (26/52) of elderly phototherapy patients had psoriasis. Psoriasis is the main indication for treatment with NB-UVB phototherapy in the general population,19 and because the risk for psoriasis increases with age,20 it is not surprising that all 3 studies found psoriasis to be the most common indication in elderly phototherapy patients. Table 3 provides further details on conditions treated in all 3 studies.
Comment
Our study found that 94% of patients with psoriasis achieved clearance with an average of 30.4 treatments, which is comparable to the reported 91% response rate with an average of 30 treatments in the United Kingdom.16 The other similar study in Turkey17 reported 73.7% of psoriasis patients achieved a 75% or more improvement from baseline with an average of 42 treatments, which may reflect underlying differences in regional skin type. Of note, the scatter chart (Figure 3) shows that several patients in the present study’s analysis are listed as not clear, but many of those patients had low treatment numbers below the mean time to clearance. Thus, the present study’s response rate may have been underestimated.
Figure 3. Comparison of total treatments and side effects across all conditions. MF indicates mycosis fungoides; DNC, did not clear. Bold rule indicates patients who experienced side effects greater than grade 1.
In the general population, studies show that psoriasis treated with standardized phototherapy protocols typically clears with an average of 20.6 treatments.21 The levels of clearance were similar in our study’s older population, but more treatments were required to achieve those results, with an average of 10 more treatments needed (an additional 3.3 weeks). Similar results were found in this sample for dermatitis and mycosis fungoides, indicating comparable clearance rates and levels but a need for more treatments to achieve similar results compared to the general population.
Additionally, in the current study more patients experienced grade 1 (mild) erythema (46%) and grade 2 erythema (25%) at some point in their treatment compared with the United Kingdom16 (1.89%) and Turkey17 (35%) studies, though these side effects did not impact the clearance rate. Interestingly, the current study’s scatter chart (Figure 3) illustrates that this side effect did not seem to increase with aging in this population. If anything, the erythema response was more prevalent in the median or younger patients in the sample. Erythema may have been due to the frequent use of photosensitizing medications in older adults in the United States, some of which typically get discontinued in patients 75 years and older (eg, statins). Other potential causes might include the use of phototype vs minimal erythema dose–driven protocols, the standard utilization of protocols originally designed for psoriasis vs other condition-specific protocols, missed treatments leading to increased sensitivity, or possibly shielding mishaps (eg, not wearing a prescribed face shield). Given the number of potential causes and the possibility of overlapping factors, careful analysis is important. With NB-UVB phototherapy, near-erythemogenic doses are optimal to achieve effective treatments, but this delicate balance may be more problematic for older adults. Future studies are needed to fully determine the factors at play for this population. In the interim, it is important for phototherapy-trained nurses to consider this risk carefully in the older population. They must follow the prescribed protocols that guide them to query patients about their responses to the prior treatment (eg, erythema, tenderness, itching), photosensitizing medications, missed treatments, and placement of shielding, and then adjust the treatment dosing accordingly.
Limitations This study had several limitations. Although clinical outcomes were recorded prospectively, the analysis was retrospective, unblinded, and not placebo controlled. It was conducted in a single organization (Group Health [now Kaiser Permanente Washington]) but did analyze data from 4 medical centers in different cities with diverse demographics and a variety of nursing staff providing the treatments. Although the vitiligo treatment protocol likely slowed the response rate for those patients with vitiligo, the numbers were small (ie, only 3 of 52 patients), so the researchers chose to include them in the current study. The sample population was relatively small, but when these data are evaluated alongside the studies in the United Kingdom16 and Turkey,17 they show a consistent picture illustrating the effectiveness and safety of phototherapy in the older population. Further epidemiologic studies could be helpful to further describe the usefulness of this modality compared with other treatments for a variety of dermatoses in this age group. Supplementary analysis specifically examining the relationship between the number and type of photosensitizing medications, frequency of erythema, and time to clearance also could be useful.
Conclusion
Older adults with a variety of dermatoses respond well to phototherapy and should have the opportunity to use it, particularly considering the potential for increased complications and costs from other treatment modalities, such as commonly used immunosuppressive pharmaceuticals. However, the current study and the comparison studies indicate that it is important to carefully consider the slower clearance rates and the potential risk for increased erythema in this population and adjust patient education and treatment dosing accordingly.
Unfortunately, many dermatology centers do not offer phototherapy because of infrastructure limitations such as space and specially trained nursing staff. Increasing accessibility of phototherapy for older adults through home treatments may be an alternative, given its effectiveness in the general population.22,23 In addition, home phototherapy may be worth pursuing for the older population considering the challenges they may face with transportation to the clinic setting and their increased risk for serious illness if exposed to infections such as COVID-19. The COVID-19 pandemic has brought to light the need for reliable, safe, and effective treatments that can be utilized in the safety of patients’ homes and should therefore be considered as an option for older adults. Issues such as mobility and cognitive decline could pose some complicating factors, but with the help of a well-trained family member or caregiver, home phototherapy could be a viable option that improves accessibility for older patients. Future research opportunities include further examination of the slower but ultimately equivalent response to phototherapy in the older population, the influence of photosensitizing medications on phototherapy effects, and the impact of phototherapy on utilization of immunosuppressive pharmaceuticals in older adults.
References
British Photodermatology Group. An appraisal of narrowband (TL-01) UVB phototherapy. British Photodermatology Group Workshop Report (April 1996). Br J Dermatol. 1997;137:327-330.
Foerster J, Boswell K, West J, et al. Narrowband UVB treatment is highly effective and causes a strong reduction in the use of steroid and other creams in psoriasis patients in clinical practice. PLoS ONE. 2017;12:e0181813. doi:10.1371/journal.pone.0181813
Fernández-Guarino M, Aboin-Gonzalez S, Barchino L, et al. Treatment of moderate and severe adult chronic atopic dermatitis with narrow-band UVB and the combination of narrow-band UVB/UVA phototherapy. Dermatol Ther. 2015;29:19-23.
Ryu HH, Choe YS, Jo S, et al. Remission period in psoriasis after multiple cycles of narrowband ultraviolet B phototherapy. J Dermatol. 2014;41:622-627.
Tintle S, Shemer A, Suárez-Fariñas M, et al. Reversal of atopic dermatitis with narrow-band UVB phototherapy and biomarkers for therapeutic response. J Allergy Clin Immunol. 2011;128:583-593.
Gambichler T, Breuckmann F, Boms S, et al. Narrowband UVB phototherapy in skin conditions beyond psoriasis. J Am Acad Dermatol. 2005;52:660-670.
Schneider LA, Hinrichs R, Scharffetter-Kochanek K. Phototherapy and photochemotherapy. Clin Dermatol. 2008;26:464-476.
Martin JA, Laube S, Edwards C, et al. Rate of acute adverse events for narrow-band UVB and psoralen-UVA phototherapy. Photodermatol Photoimmunol Photomed. 2007;23:68-72.
Mokos ZB, Jovic A, Ceovic R, et al. Therapeutic challenges in the mature patient. Clin Dermatol. 2018;36:128-139.
Di Lernia V, Goldust M. An overview of the efficacy and safety of systemic treatments for psoriasis in the elderly. Exp Opin Biol Ther. 2018;18:897-903.
Napolitano M, Balato N, Ayala F, et al. Psoriasis in elderly and non-elderly population: clinical and molecular features. G Ital Dermatol Venereol. 2016;151:587-595.
Grozdev IS, Van Voorhees AS, Gottlieb AB, et al. Psoriasis in the elderly: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol. 2011;65:537-545.
Click J, Alabaster A, Postlethwaite D, et al. Effect of availability of at-home phototherapy on the use of systemic medications for psoriasis. Photodermatol Photoimmunol Photomed. 2017;33:345-346.
Piaserico S, Conti A, Lo Console F, et al. Efficacy and safety of systemic treatments for psoriasis in elderly. Acta Derm Venereol. 2014;94:293-297.
Soliman A, Nofal E, Nofal A, et al. Combination therapy of methotrexate plus NB-UVB phototherapy is more effective than methotrexate monotherapy in the treatment of chronic plaque psoriasis. J Dermatol Treat. 2015;26:528-534.
Powell JB, Gach JE. Phototherapy in the elderly. Clin Exp Dermatol. 2015;40:605-610.
Bulur I, Erdogan HK, Aksu AE, et al. The efficacy and safety of phototherapy in geriatric patients: a retrospective study. An Bras Dermatol. 2018;93:33-38.
Madigan LM, Al-Jamal M, Hamzavi I. Exploring the gaps in the evidence-based application of narrowband UVB for the treatment of vitiligo. Photodermatol Photoimmunol Photomed. 2016;32:66-80.
Ibbotson SH. A perspective on the use of NB-UVB phototherapy vs. PUVA photochemotherapy. Front Med (Lausanne). 2018;5:184.
Bell LM, Sedlack R, Beard CM, et al. Incidence of psoriasis in Rochester, Minn, 1980-1983. Arch Dermatol. 1991;127:1184-1187.
Cameron H, Yule S, Dawe RS, et al. Review of an established UK home phototherapy service 1998-2011: improving access to a cost-effective treatment for chronic skin disease. Public Health. 2014;128:317-324.
Matthews SW, Simmer M, Williams L, et al. Transition of patients with psoriasis from office-based phototherapy to nurse-supported home phototherapy: a pilot study. JDNA. 2018;10:29-41.
References
British Photodermatology Group. An appraisal of narrowband (TL-01) UVB phototherapy. British Photodermatology Group Workshop Report (April 1996). Br J Dermatol. 1997;137:327-330.
Foerster J, Boswell K, West J, et al. Narrowband UVB treatment is highly effective and causes a strong reduction in the use of steroid and other creams in psoriasis patients in clinical practice. PLoS ONE. 2017;12:e0181813. doi:10.1371/journal.pone.0181813
Fernández-Guarino M, Aboin-Gonzalez S, Barchino L, et al. Treatment of moderate and severe adult chronic atopic dermatitis with narrow-band UVB and the combination of narrow-band UVB/UVA phototherapy. Dermatol Ther. 2015;29:19-23.
Ryu HH, Choe YS, Jo S, et al. Remission period in psoriasis after multiple cycles of narrowband ultraviolet B phototherapy. J Dermatol. 2014;41:622-627.
Tintle S, Shemer A, Suárez-Fariñas M, et al. Reversal of atopic dermatitis with narrow-band UVB phototherapy and biomarkers for therapeutic response. J Allergy Clin Immunol. 2011;128:583-593.
Gambichler T, Breuckmann F, Boms S, et al. Narrowband UVB phototherapy in skin conditions beyond psoriasis. J Am Acad Dermatol. 2005;52:660-670.
Schneider LA, Hinrichs R, Scharffetter-Kochanek K. Phototherapy and photochemotherapy. Clin Dermatol. 2008;26:464-476.
Martin JA, Laube S, Edwards C, et al. Rate of acute adverse events for narrow-band UVB and psoralen-UVA phototherapy. Photodermatol Photoimmunol Photomed. 2007;23:68-72.
Mokos ZB, Jovic A, Ceovic R, et al. Therapeutic challenges in the mature patient. Clin Dermatol. 2018;36:128-139.
Di Lernia V, Goldust M. An overview of the efficacy and safety of systemic treatments for psoriasis in the elderly. Exp Opin Biol Ther. 2018;18:897-903.
Napolitano M, Balato N, Ayala F, et al. Psoriasis in elderly and non-elderly population: clinical and molecular features. G Ital Dermatol Venereol. 2016;151:587-595.
Grozdev IS, Van Voorhees AS, Gottlieb AB, et al. Psoriasis in the elderly: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol. 2011;65:537-545.
Click J, Alabaster A, Postlethwaite D, et al. Effect of availability of at-home phototherapy on the use of systemic medications for psoriasis. Photodermatol Photoimmunol Photomed. 2017;33:345-346.
Piaserico S, Conti A, Lo Console F, et al. Efficacy and safety of systemic treatments for psoriasis in elderly. Acta Derm Venereol. 2014;94:293-297.
Soliman A, Nofal E, Nofal A, et al. Combination therapy of methotrexate plus NB-UVB phototherapy is more effective than methotrexate monotherapy in the treatment of chronic plaque psoriasis. J Dermatol Treat. 2015;26:528-534.
Powell JB, Gach JE. Phototherapy in the elderly. Clin Exp Dermatol. 2015;40:605-610.
Bulur I, Erdogan HK, Aksu AE, et al. The efficacy and safety of phototherapy in geriatric patients: a retrospective study. An Bras Dermatol. 2018;93:33-38.
Madigan LM, Al-Jamal M, Hamzavi I. Exploring the gaps in the evidence-based application of narrowband UVB for the treatment of vitiligo. Photodermatol Photoimmunol Photomed. 2016;32:66-80.
Ibbotson SH. A perspective on the use of NB-UVB phototherapy vs. PUVA photochemotherapy. Front Med (Lausanne). 2018;5:184.
Bell LM, Sedlack R, Beard CM, et al. Incidence of psoriasis in Rochester, Minn, 1980-1983. Arch Dermatol. 1991;127:1184-1187.
Cameron H, Yule S, Dawe RS, et al. Review of an established UK home phototherapy service 1998-2011: improving access to a cost-effective treatment for chronic skin disease. Public Health. 2014;128:317-324.
Matthews SW, Simmer M, Williams L, et al. Transition of patients with psoriasis from office-based phototherapy to nurse-supported home phototherapy: a pilot study. JDNA. 2018;10:29-41.
An excisional biopsy revealed a neoplasm in the dermis with focal invasion into the adjacent soft tissue (Figure 1). The tumor consisted of sheets of cells with cytoplasmic vacuoles and ductal differentiation (Figure 2), as well as cells with mild atypia, mild pleomorphism, rare mitotic figures, and abundant pale cytoplasm. Immunohistochemical staining was positive for cytokeratin (CK) 5, CK7, CK20, CK AE1/AE3, and p63 (Figure 3). The culmination of features including the large tumor size, immunohistochemical staining pattern, and mild pleomorphism with focal invasion into the soft tissue supported the diagnosis of hidradenocarcinoma.
Figure 1. Focal invasion into the adjacent soft tissue (H&E, original magnification ×40).
Figure 2. Sheets of vacuolated cells with ductal differentiation, mild cellular atypia, and a few sporadic mitotic figures (H&E, original magnification ×200).
Figure 3. A–C, Immunohistochemical staining was positive for cytokeratin AE1/AE3, p63, and cytokeratin 7, respectively (all original magnifications ×100).
Hidradenocarcinoma is an exceedingly rare malignant tumor of eccrine and/or apocrine origin.1 It accounts for less than 0.001% of all tumors and 1 of 13,000 skin biopsies.2 It usually arises in the head and neck region and most commonly affects older adults aged 50 to 70 years.3 The size of hidradenocarcinomas can vary; however, they typically are large, often growing to be greater than 5 cm in diameter.2 It tends to be an aggressive tumor that generally spreads to regional lymph nodes and distant viscera.4 Although it most commonly arises de novo, it may occasionally derive from a benign hidradenoma.1 The diagnosis of hidradenocarcinoma is made based on the tumor’s morphologic and pathologic characteristics. Histologically, it is characterized by an infiltrative and invasive proliferation of lobules made of large clear cells with atypical mitotic figures and nuclear pleomorphism as well as immunohistochemical features displaying various positive markers, such as carcinoembryonic antigen, epithelial membrane antigen, S-100 protein, and CKs AE1/AE3 and 5/6.2 Invasion of the adjacent soft tissue can be present and helps to confirm the diagnosis.
The differential diagnosis for hidradenocarcinoma primarily is the benign hidradenoma, which is similar both clinically and histologically with a few important differences. Hidradenocarcinomas often are larger and ulcerated. Histologically, they usually are more pleomorphic with the presence of mitotic figures in clear cells and tend to invade locally into the surrounding soft tissue. Other similar lesions such as spiradenoma, Merkel cell carcinoma, lymphangioma, cutaneous Crohn disease, tumors metastatic to the skin, and metastatic clear cell carcinomas originating from other organs also are included in the differential diagnosis.2
Spiradenomas are dermal tumors originating from the sweat glands. They typically present as bluish, painful, solitary nodules on the ventral surfaces of the upper body, though multiple nodules also are reported.5 Spiradenomas manifest as a central constellation of pale large cells surrounded by small, dark, basaloid cells containing hyperchromatic nuclei. The microscopic appearance of the blue basaloid cells contrasts with the clear cells seen in hidradenoma.5
Merkel cell carcinoma is a cutaneous neuroendocrine tumor affecting elderly or immunosuppressed individuals. It arises in sun-exposed areas and often is associated with Merkel cell polyomavirus infection. The histologic features display small and round cells that stain positive for CK8, CK18, CK19, and CK20 but stain negative for CK7, a marker that often is positive in hidradenocarcinoma.6
Lymphangioma, particularly cavernous lymphangioma, may resemble the gross appearance of hidradenoma/ hidradenocarcinoma. It usually presents as irregular clear blue papules and nodules in the skin and subcutaneous tissue.7 The key histopathologic finding in this tumor is the endothelium-lined channels that stain positive for D2-40, a lymphatic endothelium marker.7,8
Cutaneous Crohn disease is classified as noncaseating granulomatous skin lesions that are noncontinuous with the gastrointestinal tract.9 Clinical presentations in addition to skin edema include erythematous plaques, ulcerations, and erosions. Histopathology reveals sterile noncaseating granulomas made of Langerhans giant cells, epithelioid histocytes, and plasma cells.9
Metastatic clear cell carcinomas, such as renal cell carcinoma, can be differentiated by a history of primary carcinoma, demonstration of histologic vascular stroma, and other features related to metastatic clear cell carcinoma.2
There are no well-established therapeutic guidelines for hidradenocarcinoma. Wide local excision with margins greater than 2 cm is the preferred initial treatment and often is performed in conjunction with sentinel lymph node biopsy. External beam radiotherapy and adjunctive chemotherapy have been used for tumors that could not be surgically cleared. However, the efficacy of these treatments has not been well established.2 Targeted therapies recently have emerged as an alternative treatment choice for hidradenocarcinoma due to the utilization of immunohistochemical and genomic testing. The discovery of specific gene mutations or the expression of hormonal receptors in this tumor have paved the way for targeting HER2-expressing hidradenocarcinomas with trastuzumab and those expressing estrogen receptor with the estrogen receptor inhibitor tamoxifen.1 Epidermal growth factor receptor inhibitors and PI3K/Akt/mTOR (phosphatidylinositol-3-kinase/AKT/mammalian target of rapamycin) pathway inhibitors also have been used to target various signal transduction pathways.2
Wide excision with 2.5-cm margins was performed on our patient, and a positron emission tomography– computed tomography scan revealed no metastatic disease. She declined sentinel lymph node biopsy and additional treatment. Due to the risk for recurrence, she was monitored closely with skin examinations and positron emission tomography–computed tomography every 3 months for the first year and every 6 months thereafter. Thus far, she has had no evidence of local or regional recurrence.
References
Miller DH, Peterson JL, Buskirk SJ, et al. Management of metastatic apocrine hidradenocarcinoma with chemotherapy and radiation. Rare Tumors. 2015;7:6082.
Soni A, Bansal N, Kaushal V, et al. Current management approach to hidradenocarcinoma: a comprehensive review of the literature. Ecancermedicalscience. 2015;9:517.
Jinnah AH, Emory CL, Mai NH, et al. Hidradenocarcinoma presenting as soft tissue mass: case report with cytomorphologic description, histologic correlation, and differential diagnosis. Diagn Cytopathol. 2016;44:438-441.
Khan BM, Mansha MA, Ali N, et al. Hidradenocarcinoma: five years of local and systemic control of a rare sweat gland neoplasm with nodal metastasis. Cureus. 2018;10:E2884.
Miceli A, Ferrer-Bruker SJ. Spiradenoma. StatPearls. StatPearls Publishing; 2019.
Banks PD, Sandhu S, Gyorki DE, et al. Recent insights and advances in the management of Merkel cell carcinoma. J Oncol Pract. 2016; 12:637-646.
From the University of Illinois College of Medicine at Peoria. Dr. Kentosh is from the Department of Dermatology. Dr. Kentosh also is from the Soderstrom Skin Institute, Peoria.
The authors report no conflict of interest.
Correspondence: Jane S. Zhang, MD (jzhan28@uic.edu).
From the University of Illinois College of Medicine at Peoria. Dr. Kentosh is from the Department of Dermatology. Dr. Kentosh also is from the Soderstrom Skin Institute, Peoria.
The authors report no conflict of interest.
Correspondence: Jane S. Zhang, MD (jzhan28@uic.edu).
Author and Disclosure Information
From the University of Illinois College of Medicine at Peoria. Dr. Kentosh is from the Department of Dermatology. Dr. Kentosh also is from the Soderstrom Skin Institute, Peoria.
The authors report no conflict of interest.
Correspondence: Jane S. Zhang, MD (jzhan28@uic.edu).
An excisional biopsy revealed a neoplasm in the dermis with focal invasion into the adjacent soft tissue (Figure 1). The tumor consisted of sheets of cells with cytoplasmic vacuoles and ductal differentiation (Figure 2), as well as cells with mild atypia, mild pleomorphism, rare mitotic figures, and abundant pale cytoplasm. Immunohistochemical staining was positive for cytokeratin (CK) 5, CK7, CK20, CK AE1/AE3, and p63 (Figure 3). The culmination of features including the large tumor size, immunohistochemical staining pattern, and mild pleomorphism with focal invasion into the soft tissue supported the diagnosis of hidradenocarcinoma.
Figure 1. Focal invasion into the adjacent soft tissue (H&E, original magnification ×40).
Figure 2. Sheets of vacuolated cells with ductal differentiation, mild cellular atypia, and a few sporadic mitotic figures (H&E, original magnification ×200).
Figure 3. A–C, Immunohistochemical staining was positive for cytokeratin AE1/AE3, p63, and cytokeratin 7, respectively (all original magnifications ×100).
Hidradenocarcinoma is an exceedingly rare malignant tumor of eccrine and/or apocrine origin.1 It accounts for less than 0.001% of all tumors and 1 of 13,000 skin biopsies.2 It usually arises in the head and neck region and most commonly affects older adults aged 50 to 70 years.3 The size of hidradenocarcinomas can vary; however, they typically are large, often growing to be greater than 5 cm in diameter.2 It tends to be an aggressive tumor that generally spreads to regional lymph nodes and distant viscera.4 Although it most commonly arises de novo, it may occasionally derive from a benign hidradenoma.1 The diagnosis of hidradenocarcinoma is made based on the tumor’s morphologic and pathologic characteristics. Histologically, it is characterized by an infiltrative and invasive proliferation of lobules made of large clear cells with atypical mitotic figures and nuclear pleomorphism as well as immunohistochemical features displaying various positive markers, such as carcinoembryonic antigen, epithelial membrane antigen, S-100 protein, and CKs AE1/AE3 and 5/6.2 Invasion of the adjacent soft tissue can be present and helps to confirm the diagnosis.
The differential diagnosis for hidradenocarcinoma primarily is the benign hidradenoma, which is similar both clinically and histologically with a few important differences. Hidradenocarcinomas often are larger and ulcerated. Histologically, they usually are more pleomorphic with the presence of mitotic figures in clear cells and tend to invade locally into the surrounding soft tissue. Other similar lesions such as spiradenoma, Merkel cell carcinoma, lymphangioma, cutaneous Crohn disease, tumors metastatic to the skin, and metastatic clear cell carcinomas originating from other organs also are included in the differential diagnosis.2
Spiradenomas are dermal tumors originating from the sweat glands. They typically present as bluish, painful, solitary nodules on the ventral surfaces of the upper body, though multiple nodules also are reported.5 Spiradenomas manifest as a central constellation of pale large cells surrounded by small, dark, basaloid cells containing hyperchromatic nuclei. The microscopic appearance of the blue basaloid cells contrasts with the clear cells seen in hidradenoma.5
Merkel cell carcinoma is a cutaneous neuroendocrine tumor affecting elderly or immunosuppressed individuals. It arises in sun-exposed areas and often is associated with Merkel cell polyomavirus infection. The histologic features display small and round cells that stain positive for CK8, CK18, CK19, and CK20 but stain negative for CK7, a marker that often is positive in hidradenocarcinoma.6
Lymphangioma, particularly cavernous lymphangioma, may resemble the gross appearance of hidradenoma/ hidradenocarcinoma. It usually presents as irregular clear blue papules and nodules in the skin and subcutaneous tissue.7 The key histopathologic finding in this tumor is the endothelium-lined channels that stain positive for D2-40, a lymphatic endothelium marker.7,8
Cutaneous Crohn disease is classified as noncaseating granulomatous skin lesions that are noncontinuous with the gastrointestinal tract.9 Clinical presentations in addition to skin edema include erythematous plaques, ulcerations, and erosions. Histopathology reveals sterile noncaseating granulomas made of Langerhans giant cells, epithelioid histocytes, and plasma cells.9
Metastatic clear cell carcinomas, such as renal cell carcinoma, can be differentiated by a history of primary carcinoma, demonstration of histologic vascular stroma, and other features related to metastatic clear cell carcinoma.2
There are no well-established therapeutic guidelines for hidradenocarcinoma. Wide local excision with margins greater than 2 cm is the preferred initial treatment and often is performed in conjunction with sentinel lymph node biopsy. External beam radiotherapy and adjunctive chemotherapy have been used for tumors that could not be surgically cleared. However, the efficacy of these treatments has not been well established.2 Targeted therapies recently have emerged as an alternative treatment choice for hidradenocarcinoma due to the utilization of immunohistochemical and genomic testing. The discovery of specific gene mutations or the expression of hormonal receptors in this tumor have paved the way for targeting HER2-expressing hidradenocarcinomas with trastuzumab and those expressing estrogen receptor with the estrogen receptor inhibitor tamoxifen.1 Epidermal growth factor receptor inhibitors and PI3K/Akt/mTOR (phosphatidylinositol-3-kinase/AKT/mammalian target of rapamycin) pathway inhibitors also have been used to target various signal transduction pathways.2
Wide excision with 2.5-cm margins was performed on our patient, and a positron emission tomography– computed tomography scan revealed no metastatic disease. She declined sentinel lymph node biopsy and additional treatment. Due to the risk for recurrence, she was monitored closely with skin examinations and positron emission tomography–computed tomography every 3 months for the first year and every 6 months thereafter. Thus far, she has had no evidence of local or regional recurrence.
The Diagnosis: Hidradenocarcinoma
An excisional biopsy revealed a neoplasm in the dermis with focal invasion into the adjacent soft tissue (Figure 1). The tumor consisted of sheets of cells with cytoplasmic vacuoles and ductal differentiation (Figure 2), as well as cells with mild atypia, mild pleomorphism, rare mitotic figures, and abundant pale cytoplasm. Immunohistochemical staining was positive for cytokeratin (CK) 5, CK7, CK20, CK AE1/AE3, and p63 (Figure 3). The culmination of features including the large tumor size, immunohistochemical staining pattern, and mild pleomorphism with focal invasion into the soft tissue supported the diagnosis of hidradenocarcinoma.
Figure 1. Focal invasion into the adjacent soft tissue (H&E, original magnification ×40).
Figure 2. Sheets of vacuolated cells with ductal differentiation, mild cellular atypia, and a few sporadic mitotic figures (H&E, original magnification ×200).
Figure 3. A–C, Immunohistochemical staining was positive for cytokeratin AE1/AE3, p63, and cytokeratin 7, respectively (all original magnifications ×100).
Hidradenocarcinoma is an exceedingly rare malignant tumor of eccrine and/or apocrine origin.1 It accounts for less than 0.001% of all tumors and 1 of 13,000 skin biopsies.2 It usually arises in the head and neck region and most commonly affects older adults aged 50 to 70 years.3 The size of hidradenocarcinomas can vary; however, they typically are large, often growing to be greater than 5 cm in diameter.2 It tends to be an aggressive tumor that generally spreads to regional lymph nodes and distant viscera.4 Although it most commonly arises de novo, it may occasionally derive from a benign hidradenoma.1 The diagnosis of hidradenocarcinoma is made based on the tumor’s morphologic and pathologic characteristics. Histologically, it is characterized by an infiltrative and invasive proliferation of lobules made of large clear cells with atypical mitotic figures and nuclear pleomorphism as well as immunohistochemical features displaying various positive markers, such as carcinoembryonic antigen, epithelial membrane antigen, S-100 protein, and CKs AE1/AE3 and 5/6.2 Invasion of the adjacent soft tissue can be present and helps to confirm the diagnosis.
The differential diagnosis for hidradenocarcinoma primarily is the benign hidradenoma, which is similar both clinically and histologically with a few important differences. Hidradenocarcinomas often are larger and ulcerated. Histologically, they usually are more pleomorphic with the presence of mitotic figures in clear cells and tend to invade locally into the surrounding soft tissue. Other similar lesions such as spiradenoma, Merkel cell carcinoma, lymphangioma, cutaneous Crohn disease, tumors metastatic to the skin, and metastatic clear cell carcinomas originating from other organs also are included in the differential diagnosis.2
Spiradenomas are dermal tumors originating from the sweat glands. They typically present as bluish, painful, solitary nodules on the ventral surfaces of the upper body, though multiple nodules also are reported.5 Spiradenomas manifest as a central constellation of pale large cells surrounded by small, dark, basaloid cells containing hyperchromatic nuclei. The microscopic appearance of the blue basaloid cells contrasts with the clear cells seen in hidradenoma.5
Merkel cell carcinoma is a cutaneous neuroendocrine tumor affecting elderly or immunosuppressed individuals. It arises in sun-exposed areas and often is associated with Merkel cell polyomavirus infection. The histologic features display small and round cells that stain positive for CK8, CK18, CK19, and CK20 but stain negative for CK7, a marker that often is positive in hidradenocarcinoma.6
Lymphangioma, particularly cavernous lymphangioma, may resemble the gross appearance of hidradenoma/ hidradenocarcinoma. It usually presents as irregular clear blue papules and nodules in the skin and subcutaneous tissue.7 The key histopathologic finding in this tumor is the endothelium-lined channels that stain positive for D2-40, a lymphatic endothelium marker.7,8
Cutaneous Crohn disease is classified as noncaseating granulomatous skin lesions that are noncontinuous with the gastrointestinal tract.9 Clinical presentations in addition to skin edema include erythematous plaques, ulcerations, and erosions. Histopathology reveals sterile noncaseating granulomas made of Langerhans giant cells, epithelioid histocytes, and plasma cells.9
Metastatic clear cell carcinomas, such as renal cell carcinoma, can be differentiated by a history of primary carcinoma, demonstration of histologic vascular stroma, and other features related to metastatic clear cell carcinoma.2
There are no well-established therapeutic guidelines for hidradenocarcinoma. Wide local excision with margins greater than 2 cm is the preferred initial treatment and often is performed in conjunction with sentinel lymph node biopsy. External beam radiotherapy and adjunctive chemotherapy have been used for tumors that could not be surgically cleared. However, the efficacy of these treatments has not been well established.2 Targeted therapies recently have emerged as an alternative treatment choice for hidradenocarcinoma due to the utilization of immunohistochemical and genomic testing. The discovery of specific gene mutations or the expression of hormonal receptors in this tumor have paved the way for targeting HER2-expressing hidradenocarcinomas with trastuzumab and those expressing estrogen receptor with the estrogen receptor inhibitor tamoxifen.1 Epidermal growth factor receptor inhibitors and PI3K/Akt/mTOR (phosphatidylinositol-3-kinase/AKT/mammalian target of rapamycin) pathway inhibitors also have been used to target various signal transduction pathways.2
Wide excision with 2.5-cm margins was performed on our patient, and a positron emission tomography– computed tomography scan revealed no metastatic disease. She declined sentinel lymph node biopsy and additional treatment. Due to the risk for recurrence, she was monitored closely with skin examinations and positron emission tomography–computed tomography every 3 months for the first year and every 6 months thereafter. Thus far, she has had no evidence of local or regional recurrence.
References
Miller DH, Peterson JL, Buskirk SJ, et al. Management of metastatic apocrine hidradenocarcinoma with chemotherapy and radiation. Rare Tumors. 2015;7:6082.
Soni A, Bansal N, Kaushal V, et al. Current management approach to hidradenocarcinoma: a comprehensive review of the literature. Ecancermedicalscience. 2015;9:517.
Jinnah AH, Emory CL, Mai NH, et al. Hidradenocarcinoma presenting as soft tissue mass: case report with cytomorphologic description, histologic correlation, and differential diagnosis. Diagn Cytopathol. 2016;44:438-441.
Khan BM, Mansha MA, Ali N, et al. Hidradenocarcinoma: five years of local and systemic control of a rare sweat gland neoplasm with nodal metastasis. Cureus. 2018;10:E2884.
Miceli A, Ferrer-Bruker SJ. Spiradenoma. StatPearls. StatPearls Publishing; 2019.
Banks PD, Sandhu S, Gyorki DE, et al. Recent insights and advances in the management of Merkel cell carcinoma. J Oncol Pract. 2016; 12:637-646.
Kalof AN, Cooper K. D2-40 immunohistochemistry—so far! Adv Anat Pathol. 2009;16:62-64.
Schneider SL, Foster K, Patel D, et al. Cutaneous manifestations of metastatic Crohn’s disease. Pediatr Dermatol. 2018;35:566-574.
References
Miller DH, Peterson JL, Buskirk SJ, et al. Management of metastatic apocrine hidradenocarcinoma with chemotherapy and radiation. Rare Tumors. 2015;7:6082.
Soni A, Bansal N, Kaushal V, et al. Current management approach to hidradenocarcinoma: a comprehensive review of the literature. Ecancermedicalscience. 2015;9:517.
Jinnah AH, Emory CL, Mai NH, et al. Hidradenocarcinoma presenting as soft tissue mass: case report with cytomorphologic description, histologic correlation, and differential diagnosis. Diagn Cytopathol. 2016;44:438-441.
Khan BM, Mansha MA, Ali N, et al. Hidradenocarcinoma: five years of local and systemic control of a rare sweat gland neoplasm with nodal metastasis. Cureus. 2018;10:E2884.
Miceli A, Ferrer-Bruker SJ. Spiradenoma. StatPearls. StatPearls Publishing; 2019.
Banks PD, Sandhu S, Gyorki DE, et al. Recent insights and advances in the management of Merkel cell carcinoma. J Oncol Pract. 2016; 12:637-646.
A 20-year-old woman with no notable medical history presented to the dermatology clinic with an enlarging mass on the right buttock that had been growing over the course of several years. The mass progressed from a small, mildly tender nodule to a 10×10-cm, hyperpigmented, exophytic tumor. There were no other abnormal findings on physical examination, and the patient denied any systemic symptoms.
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Traction alopecia (TA)--one of the most common types of hair loss in Black women (although not exclusive to Black women)--is reversible when early corrective measures are taken; if chronic tension continues, however, permanent scarring alopecia ensues. Dermatologists can prevent worsening of this distressing hair loss. Due to a dearth of training among dermatologists in conditions occurring in patients with tightly coiled hair, it is imperative to add practical methods to the body of dermatology literature, with the goal of enhancing cultural humility.
Hairstyling among Black women often is a lengthy process and often results in relationship bonding with the hair care giver, in turn imparting hair care traditions to the next generation. Therefore, a well-received discussion about TA prevention not only has an impact on the patient but potentially on a multigenerational family of women and friends. We present a memory aid for discussing TA, with a focus on cultural humility and patient-centered communication.
Factors contributing to the risk of TA are hairstyles and hair care practices commonly used in Black individuals, including braids, locs, weaves, wigs, and chemical straightening.1 These styles often are worn to increase hair manageability or as a creative expression of beauty.
Discussing TA can be distressing for physicians and patients, especially in the setting of hair texture discordance. In a study that surveyed Black patients' perception of their dermatologic care both in and outside of a skin of color clinic, 71% of respondents (12/17) said that they prefer a race-concordant dermatologist. Some respondents reported that non-skin of color clinic dermatologists examined their hair with the end of a pencil or not at all; patients interpreted these interactions as disrespectful and racially insensitive.2 Another study found that only 30.2% (19/63) of dermatology chief residents and 12.2% (5/41) of program directors reported a specific rotation during which residents gained experience treating skin of color patients.3
Due to a paucity of training in diagnosing and treating patients with tightly coiled hair who experience hair loss, some physicians might feel uncomfortable caring for patients who have tightly coiled hair. Although many Black patients prefer to see a race-concordant dermatologist because of their perceived cultural competence and shared experience, there is a paucity of Black dermatologists to see all patients who have tightly coiled hair.4 Therefore, all dermatologists should become skilled and comfortable discussing and treating TA in patients with all hair types.
METHOD FOR COUNSELING
The following scenarios are a guide to begin closing the competency gap in counseling about TA, using a "compliment, discuss, and suggest" method.
Scenario 1 A Black woman presents with a concern of "thinning edges" (a popular term on social media for TA). A hair-discordant dermatologist tells her, first, that she has TA caused by wearing tight hairstyles and, second, that the treatment is to stop wearing tight braids and weaves and to discontinue chemical relaxers. The dermatologist then leaves the room.
The Patient's Perspective It is not uncommon for the patient to have feelings of frustration about how they will style their hair, especially if they are unfamiliar with caring for their hair in its natural state.5 Also, they might have feelings of dismay that the loving childhood hair care giver, often their mother or grandmother, unintentionally harmed them with a tight style. They also might feel betrayed by their hairstylist, who might not have encouraged them to see a dermatologist, or who continued to oblige their request for a high-risk hairstyle. The patient might feel uncomfortable communicating the dermatologist's new recommendations to their hair care team, who also are part of her emotional support system. The patient also might think that the hair-discordant dermatologist has no idea what they "go through" with their hair.
"Compliment, Discuss, and Suggest" Counseling Traction alopecia is caused by tight hairstyles that often hurt when they are put in as tight braids, weaves, and ponytails.6 Risk increases if tight styles are applied to chemically straightened hair.1 Braids, sew-in weaves, and wigs with adhesive sometimes are referred to as protective styles. However, these styles can still lead to TA due to excessive tension.
Compliment: "Your hair looks great. I know that you get many compliments."
Discuss: "However, some of the styles might be increasing your risk for hair loss. Our goal is to preserve as many of your follicles as possible."
Suggest: "Let's start by loosening the hairstyle if it is painful when being applied. Pain means inflammation, which can lead to scarring of hair follicles and worsening of hair loss."
Using pronouns such as we, us, and our is intentional. Doing so signals that the dermatologist is a partner with the patient in the treatment of TA. Starting with a simple initial recommendation gives the patient time to process the common thoughts highlighted in The Patient's Perspective section.6
Scenario 2 A Black child (we'll call her "Janet") is accompanied by her mother for follow-up of mild atopic dermatitis on the body and scalp. When the dermatologist examines the patient's scalp, they note that she has the fringe sign--retained short hairs along the frontal hairline--that is consistent with TA. Janet's hair is adorned with 2 tight ponytails in the front with colorful decorative balls on ponytail ties, barrettes, and 6 cornrow braids in the back with plastic beads on the ends. The dermatologist counsels about the atopic dermatitis and leaves the room.
"Compliment, Discuss, and Suggest" Counseling The use of tight decorative balls on ponytail ties and numerous plastic beads increases the amount of tension and weight on the hair, which may lead to a higher risk for developing traction alopecia.6 It is quite common for children of African descent to wear hair adornments. Proper counseling regarding their use and possible implications is essential.
Compliment: "You're doing a great job controlling the atopic dermatitis, which can cause Janet's scalp to be dry. Also, her hair is beautiful--it looks like you spent a lot of time on her hair. And Janet, I like the color of your barrettes."
Discuss: "Mom, I just noticed that a few areas look tight. Let's look together." (The dermatologist points out areas where the scalp is tented upward due to traction, follicular pustules or papules, or the frontal fringe sign.) "I'm on a mission to #savetheedges because we want Janet to grow up with full edges." (Again, loss of "edges" refers to TA.)
Suggest: "When you do Janet's hair, it's OK if every hair is not in place. In fact, making styles look and feel 1 or 2 weeks old will lessen tension on the scalp. Remove Janet's hair ties to release tension when she is at home and while she's sleeping, if possible. Every minute that the hair is loose really does help."6
The Parent's Perspective
All parents take pride in their children. In some Black communities, mothers are judged by how well they manage and style their children's hair. Some people might even suggest that parents of children with nonstyled, tightly coiled hair are not fit parents. Anthropologist Sylvia Boone, PhD, found that among the Mende tribe in Sierra Leone, "unkempt, 'neglected,' or 'messy' hair implied that a woman either had loose morals or was insane."7
Braids are commonly worn by people of African heritage for a variety of reasons, including ease of manageability, to decrease daily hairstyling time, and as an expression of creativity. Intricate neat hairstyles, despite the risk of pain and TA, are perceived as a sign that the child is cared for and loved.6
FINAL THOUGHTS
Patient-centered communication is associated with the patient trusting the physician, which is especially important in race-discordant physician-patient relationships. A study found that patient-physician race discordance led to shorter visits, a lower rating of patient affect, and less shared decision-making.8 Moreover, in a study of primary care clinicians, implicit bias was found to affect communication patterns and social interactions, impacting patient outcomes. Downstream effects of racial bias resulted in less speaking, smiling, and social comments when interacting with Black patients.9
These findings highlight the need to address interpersonal barriers to effective communication in race-discordant patient-physician dyads. A history of segregated neighborhoods and schools might contribute to structural barriers, resulting in lack of familiarity with cultural norms outside one's culture, which might globally perpetuate poor communication and patient outcomes.
The "compliment, discuss, and suggest" method might lead to more positive physician-patient encounters by having the dermatologist focus on empathetically understanding the patient's perspective.10 Effective communication, understanding cultural hair care practices, and a thorough scalp examination are paramount for patients with tightly coiled hair.11 Early intervention in TA is crucial and involves partnering with patients and parents to amend high-risk hairstyling routines with cultural humility.
Dr. Grayson is from the Florida State University College of Medicine Internal Medicine Residency Program, Tallahassee. Dr. Heath is from the Department of Dermatology, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania.
The authors report no conflict of interest.
Correspondence: Candrice R. Heath, MD, 3401 N Broad St, 5OB, Philadelphia, PA 19140 (Candrice.Heath@tuhs.temple.edu).
Dr. Grayson is from the Florida State University College of Medicine Internal Medicine Residency Program, Tallahassee. Dr. Heath is from the Department of Dermatology, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania.
The authors report no conflict of interest.
Correspondence: Candrice R. Heath, MD, 3401 N Broad St, 5OB, Philadelphia, PA 19140 (Candrice.Heath@tuhs.temple.edu).
Author and Disclosure Information
Dr. Grayson is from the Florida State University College of Medicine Internal Medicine Residency Program, Tallahassee. Dr. Heath is from the Department of Dermatology, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania.
The authors report no conflict of interest.
Correspondence: Candrice R. Heath, MD, 3401 N Broad St, 5OB, Philadelphia, PA 19140 (Candrice.Heath@tuhs.temple.edu).
Traction alopecia (TA)--one of the most common types of hair loss in Black women (although not exclusive to Black women)--is reversible when early corrective measures are taken; if chronic tension continues, however, permanent scarring alopecia ensues. Dermatologists can prevent worsening of this distressing hair loss. Due to a dearth of training among dermatologists in conditions occurring in patients with tightly coiled hair, it is imperative to add practical methods to the body of dermatology literature, with the goal of enhancing cultural humility.
Hairstyling among Black women often is a lengthy process and often results in relationship bonding with the hair care giver, in turn imparting hair care traditions to the next generation. Therefore, a well-received discussion about TA prevention not only has an impact on the patient but potentially on a multigenerational family of women and friends. We present a memory aid for discussing TA, with a focus on cultural humility and patient-centered communication.
Factors contributing to the risk of TA are hairstyles and hair care practices commonly used in Black individuals, including braids, locs, weaves, wigs, and chemical straightening.1 These styles often are worn to increase hair manageability or as a creative expression of beauty.
Discussing TA can be distressing for physicians and patients, especially in the setting of hair texture discordance. In a study that surveyed Black patients' perception of their dermatologic care both in and outside of a skin of color clinic, 71% of respondents (12/17) said that they prefer a race-concordant dermatologist. Some respondents reported that non-skin of color clinic dermatologists examined their hair with the end of a pencil or not at all; patients interpreted these interactions as disrespectful and racially insensitive.2 Another study found that only 30.2% (19/63) of dermatology chief residents and 12.2% (5/41) of program directors reported a specific rotation during which residents gained experience treating skin of color patients.3
Due to a paucity of training in diagnosing and treating patients with tightly coiled hair who experience hair loss, some physicians might feel uncomfortable caring for patients who have tightly coiled hair. Although many Black patients prefer to see a race-concordant dermatologist because of their perceived cultural competence and shared experience, there is a paucity of Black dermatologists to see all patients who have tightly coiled hair.4 Therefore, all dermatologists should become skilled and comfortable discussing and treating TA in patients with all hair types.
METHOD FOR COUNSELING
The following scenarios are a guide to begin closing the competency gap in counseling about TA, using a "compliment, discuss, and suggest" method.
Scenario 1 A Black woman presents with a concern of "thinning edges" (a popular term on social media for TA). A hair-discordant dermatologist tells her, first, that she has TA caused by wearing tight hairstyles and, second, that the treatment is to stop wearing tight braids and weaves and to discontinue chemical relaxers. The dermatologist then leaves the room.
The Patient's Perspective It is not uncommon for the patient to have feelings of frustration about how they will style their hair, especially if they are unfamiliar with caring for their hair in its natural state.5 Also, they might have feelings of dismay that the loving childhood hair care giver, often their mother or grandmother, unintentionally harmed them with a tight style. They also might feel betrayed by their hairstylist, who might not have encouraged them to see a dermatologist, or who continued to oblige their request for a high-risk hairstyle. The patient might feel uncomfortable communicating the dermatologist's new recommendations to their hair care team, who also are part of her emotional support system. The patient also might think that the hair-discordant dermatologist has no idea what they "go through" with their hair.
"Compliment, Discuss, and Suggest" Counseling Traction alopecia is caused by tight hairstyles that often hurt when they are put in as tight braids, weaves, and ponytails.6 Risk increases if tight styles are applied to chemically straightened hair.1 Braids, sew-in weaves, and wigs with adhesive sometimes are referred to as protective styles. However, these styles can still lead to TA due to excessive tension.
Compliment: "Your hair looks great. I know that you get many compliments."
Discuss: "However, some of the styles might be increasing your risk for hair loss. Our goal is to preserve as many of your follicles as possible."
Suggest: "Let's start by loosening the hairstyle if it is painful when being applied. Pain means inflammation, which can lead to scarring of hair follicles and worsening of hair loss."
Using pronouns such as we, us, and our is intentional. Doing so signals that the dermatologist is a partner with the patient in the treatment of TA. Starting with a simple initial recommendation gives the patient time to process the common thoughts highlighted in The Patient's Perspective section.6
Scenario 2 A Black child (we'll call her "Janet") is accompanied by her mother for follow-up of mild atopic dermatitis on the body and scalp. When the dermatologist examines the patient's scalp, they note that she has the fringe sign--retained short hairs along the frontal hairline--that is consistent with TA. Janet's hair is adorned with 2 tight ponytails in the front with colorful decorative balls on ponytail ties, barrettes, and 6 cornrow braids in the back with plastic beads on the ends. The dermatologist counsels about the atopic dermatitis and leaves the room.
"Compliment, Discuss, and Suggest" Counseling The use of tight decorative balls on ponytail ties and numerous plastic beads increases the amount of tension and weight on the hair, which may lead to a higher risk for developing traction alopecia.6 It is quite common for children of African descent to wear hair adornments. Proper counseling regarding their use and possible implications is essential.
Compliment: "You're doing a great job controlling the atopic dermatitis, which can cause Janet's scalp to be dry. Also, her hair is beautiful--it looks like you spent a lot of time on her hair. And Janet, I like the color of your barrettes."
Discuss: "Mom, I just noticed that a few areas look tight. Let's look together." (The dermatologist points out areas where the scalp is tented upward due to traction, follicular pustules or papules, or the frontal fringe sign.) "I'm on a mission to #savetheedges because we want Janet to grow up with full edges." (Again, loss of "edges" refers to TA.)
Suggest: "When you do Janet's hair, it's OK if every hair is not in place. In fact, making styles look and feel 1 or 2 weeks old will lessen tension on the scalp. Remove Janet's hair ties to release tension when she is at home and while she's sleeping, if possible. Every minute that the hair is loose really does help."6
The Parent's Perspective
All parents take pride in their children. In some Black communities, mothers are judged by how well they manage and style their children's hair. Some people might even suggest that parents of children with nonstyled, tightly coiled hair are not fit parents. Anthropologist Sylvia Boone, PhD, found that among the Mende tribe in Sierra Leone, "unkempt, 'neglected,' or 'messy' hair implied that a woman either had loose morals or was insane."7
Braids are commonly worn by people of African heritage for a variety of reasons, including ease of manageability, to decrease daily hairstyling time, and as an expression of creativity. Intricate neat hairstyles, despite the risk of pain and TA, are perceived as a sign that the child is cared for and loved.6
FINAL THOUGHTS
Patient-centered communication is associated with the patient trusting the physician, which is especially important in race-discordant physician-patient relationships. A study found that patient-physician race discordance led to shorter visits, a lower rating of patient affect, and less shared decision-making.8 Moreover, in a study of primary care clinicians, implicit bias was found to affect communication patterns and social interactions, impacting patient outcomes. Downstream effects of racial bias resulted in less speaking, smiling, and social comments when interacting with Black patients.9
These findings highlight the need to address interpersonal barriers to effective communication in race-discordant patient-physician dyads. A history of segregated neighborhoods and schools might contribute to structural barriers, resulting in lack of familiarity with cultural norms outside one's culture, which might globally perpetuate poor communication and patient outcomes.
The "compliment, discuss, and suggest" method might lead to more positive physician-patient encounters by having the dermatologist focus on empathetically understanding the patient's perspective.10 Effective communication, understanding cultural hair care practices, and a thorough scalp examination are paramount for patients with tightly coiled hair.11 Early intervention in TA is crucial and involves partnering with patients and parents to amend high-risk hairstyling routines with cultural humility.
Traction alopecia (TA)--one of the most common types of hair loss in Black women (although not exclusive to Black women)--is reversible when early corrective measures are taken; if chronic tension continues, however, permanent scarring alopecia ensues. Dermatologists can prevent worsening of this distressing hair loss. Due to a dearth of training among dermatologists in conditions occurring in patients with tightly coiled hair, it is imperative to add practical methods to the body of dermatology literature, with the goal of enhancing cultural humility.
Hairstyling among Black women often is a lengthy process and often results in relationship bonding with the hair care giver, in turn imparting hair care traditions to the next generation. Therefore, a well-received discussion about TA prevention not only has an impact on the patient but potentially on a multigenerational family of women and friends. We present a memory aid for discussing TA, with a focus on cultural humility and patient-centered communication.
Factors contributing to the risk of TA are hairstyles and hair care practices commonly used in Black individuals, including braids, locs, weaves, wigs, and chemical straightening.1 These styles often are worn to increase hair manageability or as a creative expression of beauty.
Discussing TA can be distressing for physicians and patients, especially in the setting of hair texture discordance. In a study that surveyed Black patients' perception of their dermatologic care both in and outside of a skin of color clinic, 71% of respondents (12/17) said that they prefer a race-concordant dermatologist. Some respondents reported that non-skin of color clinic dermatologists examined their hair with the end of a pencil or not at all; patients interpreted these interactions as disrespectful and racially insensitive.2 Another study found that only 30.2% (19/63) of dermatology chief residents and 12.2% (5/41) of program directors reported a specific rotation during which residents gained experience treating skin of color patients.3
Due to a paucity of training in diagnosing and treating patients with tightly coiled hair who experience hair loss, some physicians might feel uncomfortable caring for patients who have tightly coiled hair. Although many Black patients prefer to see a race-concordant dermatologist because of their perceived cultural competence and shared experience, there is a paucity of Black dermatologists to see all patients who have tightly coiled hair.4 Therefore, all dermatologists should become skilled and comfortable discussing and treating TA in patients with all hair types.
METHOD FOR COUNSELING
The following scenarios are a guide to begin closing the competency gap in counseling about TA, using a "compliment, discuss, and suggest" method.
Scenario 1 A Black woman presents with a concern of "thinning edges" (a popular term on social media for TA). A hair-discordant dermatologist tells her, first, that she has TA caused by wearing tight hairstyles and, second, that the treatment is to stop wearing tight braids and weaves and to discontinue chemical relaxers. The dermatologist then leaves the room.
The Patient's Perspective It is not uncommon for the patient to have feelings of frustration about how they will style their hair, especially if they are unfamiliar with caring for their hair in its natural state.5 Also, they might have feelings of dismay that the loving childhood hair care giver, often their mother or grandmother, unintentionally harmed them with a tight style. They also might feel betrayed by their hairstylist, who might not have encouraged them to see a dermatologist, or who continued to oblige their request for a high-risk hairstyle. The patient might feel uncomfortable communicating the dermatologist's new recommendations to their hair care team, who also are part of her emotional support system. The patient also might think that the hair-discordant dermatologist has no idea what they "go through" with their hair.
"Compliment, Discuss, and Suggest" Counseling Traction alopecia is caused by tight hairstyles that often hurt when they are put in as tight braids, weaves, and ponytails.6 Risk increases if tight styles are applied to chemically straightened hair.1 Braids, sew-in weaves, and wigs with adhesive sometimes are referred to as protective styles. However, these styles can still lead to TA due to excessive tension.
Compliment: "Your hair looks great. I know that you get many compliments."
Discuss: "However, some of the styles might be increasing your risk for hair loss. Our goal is to preserve as many of your follicles as possible."
Suggest: "Let's start by loosening the hairstyle if it is painful when being applied. Pain means inflammation, which can lead to scarring of hair follicles and worsening of hair loss."
Using pronouns such as we, us, and our is intentional. Doing so signals that the dermatologist is a partner with the patient in the treatment of TA. Starting with a simple initial recommendation gives the patient time to process the common thoughts highlighted in The Patient's Perspective section.6
Scenario 2 A Black child (we'll call her "Janet") is accompanied by her mother for follow-up of mild atopic dermatitis on the body and scalp. When the dermatologist examines the patient's scalp, they note that she has the fringe sign--retained short hairs along the frontal hairline--that is consistent with TA. Janet's hair is adorned with 2 tight ponytails in the front with colorful decorative balls on ponytail ties, barrettes, and 6 cornrow braids in the back with plastic beads on the ends. The dermatologist counsels about the atopic dermatitis and leaves the room.
"Compliment, Discuss, and Suggest" Counseling The use of tight decorative balls on ponytail ties and numerous plastic beads increases the amount of tension and weight on the hair, which may lead to a higher risk for developing traction alopecia.6 It is quite common for children of African descent to wear hair adornments. Proper counseling regarding their use and possible implications is essential.
Compliment: "You're doing a great job controlling the atopic dermatitis, which can cause Janet's scalp to be dry. Also, her hair is beautiful--it looks like you spent a lot of time on her hair. And Janet, I like the color of your barrettes."
Discuss: "Mom, I just noticed that a few areas look tight. Let's look together." (The dermatologist points out areas where the scalp is tented upward due to traction, follicular pustules or papules, or the frontal fringe sign.) "I'm on a mission to #savetheedges because we want Janet to grow up with full edges." (Again, loss of "edges" refers to TA.)
Suggest: "When you do Janet's hair, it's OK if every hair is not in place. In fact, making styles look and feel 1 or 2 weeks old will lessen tension on the scalp. Remove Janet's hair ties to release tension when she is at home and while she's sleeping, if possible. Every minute that the hair is loose really does help."6
The Parent's Perspective
All parents take pride in their children. In some Black communities, mothers are judged by how well they manage and style their children's hair. Some people might even suggest that parents of children with nonstyled, tightly coiled hair are not fit parents. Anthropologist Sylvia Boone, PhD, found that among the Mende tribe in Sierra Leone, "unkempt, 'neglected,' or 'messy' hair implied that a woman either had loose morals or was insane."7
Braids are commonly worn by people of African heritage for a variety of reasons, including ease of manageability, to decrease daily hairstyling time, and as an expression of creativity. Intricate neat hairstyles, despite the risk of pain and TA, are perceived as a sign that the child is cared for and loved.6
FINAL THOUGHTS
Patient-centered communication is associated with the patient trusting the physician, which is especially important in race-discordant physician-patient relationships. A study found that patient-physician race discordance led to shorter visits, a lower rating of patient affect, and less shared decision-making.8 Moreover, in a study of primary care clinicians, implicit bias was found to affect communication patterns and social interactions, impacting patient outcomes. Downstream effects of racial bias resulted in less speaking, smiling, and social comments when interacting with Black patients.9
These findings highlight the need to address interpersonal barriers to effective communication in race-discordant patient-physician dyads. A history of segregated neighborhoods and schools might contribute to structural barriers, resulting in lack of familiarity with cultural norms outside one's culture, which might globally perpetuate poor communication and patient outcomes.
The "compliment, discuss, and suggest" method might lead to more positive physician-patient encounters by having the dermatologist focus on empathetically understanding the patient's perspective.10 Effective communication, understanding cultural hair care practices, and a thorough scalp examination are paramount for patients with tightly coiled hair.11 Early intervention in TA is crucial and involves partnering with patients and parents to amend high-risk hairstyling routines with cultural humility.
A Elbow and forearm with erythematous, well-demarcated, pink plaques with mild micaceous scale in a 42-year-old White woman.
B Elbow and forearm with violaceous, well-demarcated plaques with micaceous scale and hyperpigmented patches around the active plaques in a 58-year-old Black man.
Epidemiology Psoriasis prevalence in the United States has been estimated at 3.7%.1-3 If broken down by race or ethnicity, the prevalence of psoriasis varies: 2.5% to 3.7% in White adults1-4; 1.3% to 2% in Black adults1-4; 1.6% in Hispanics/ other adults1-3; 1% in children overall; 0.29% in White children1,5; and 0.06% in Black children.1,5
Key clinical features in people with darker skin tones include:
plaques that may appear more violaceous in color instead of pink or erythematous
higher body surface area of involvement4 and thicker, more scaly plaques6
increased likelihood of postinflammatory hyperpigmentation (PIH).
Worth noting Although individuals of all skin tones may experience the psychosocial impact of psoriasis, quality-of-life measures have been found to be worse in those with skin of color (SOC) compared to White patients.1,4 This may be due to the lingering PIH and hypopigmentation that occurs even after inflammatory plaques are treated. Of course, lack of access to care contributes to greater disease burden and more devastating psychological impact.
Health disparity highlight Psoriasis may be underreported and underdiagnosed in individuals with SOC, as factors contributing to health care disparities may play a role, such as access to health care in general,1,7 and access to clinicians proficient in diagnosing cutaneous diseases in SOC may be delayed.8
Biologic medications are used less often in Black patients than in White patients, despite biologic medications being very efficacious for treatment of psoriasis.1,9,10
References
Kaufman BP, Alexis AF. Psoriasis in skin of color: insights into the epidemiology, clinical presentation, genetics, quality-of-life impact, and treatment of psoriasis in non-white racial/ethnic groups. Am J Clin Dermatol. 2018;19:405-423.
Rachakonda TD, Schupp CW, Armstrong AW. Psoriasis prevalence among adults in the United States. J Am Acad Dermatol. 2014;70:512-516.
Helmick CG, Lee-Han H, Hirsch SC, et al. Prevalence of psoriasis among adults in the U.S.: 2003-2006 and 2009-2010 National Health and Nutrition Examination Surveys. Am J Prev Med. 2014;47:37-45.
Gelfand JM, Stern RS, Nijsten T, et al. The prevalence of psoriasis in African Americans: results from a population-based study. J Am Acad Dermatol. 2005;52:23-26.
Wu JJ, Black MH, Smith N, et al. Low prevalence of psoriasis among children and adolescents in a large multiethnic cohort in southern California. J Am Acad Dermatol. 2011;65:957-964.
Davis SA, Narahari S, Feldman SR, et al. Top dermatologic conditions in patients of color: an analysis of nationally representative data. J Drugs Dermatol. 2012;11:466-473.
Alexis AF, Blackcloud P. Psoriasis in skin of color: epidemiology, genetics, clinical presentation, and treatment nuances. J Clin Aesthet Dermatol. 2014;7:16-24.
Mundluru SN, Ramalingam ND, Tran HN. Addressing internal medicine residents’ discomfort with basic dermatology in persons of color in the primary care clinic. Am J Med Qual. 2019;34:513-513.
Kerr GS, Qaiyumi S, Richards J, et al. Psoriasis and psoriatic arthritis in African-American patients—the need to measure disease burden. Clin Rheumatol. 2015;34:1753-1759.
Takeshita J, Gelfand JM, Li P, et al. Psoriasis in the US Medicare population: prevalence, treatment, and factors associated with biologic use. J Invest Dermatol. 2015;135:2955-2963.
A Elbow and forearm with erythematous, well-demarcated, pink plaques with mild micaceous scale in a 42-year-old White woman.
B Elbow and forearm with violaceous, well-demarcated plaques with micaceous scale and hyperpigmented patches around the active plaques in a 58-year-old Black man.
Epidemiology Psoriasis prevalence in the United States has been estimated at 3.7%.1-3 If broken down by race or ethnicity, the prevalence of psoriasis varies: 2.5% to 3.7% in White adults1-4; 1.3% to 2% in Black adults1-4; 1.6% in Hispanics/ other adults1-3; 1% in children overall; 0.29% in White children1,5; and 0.06% in Black children.1,5
Key clinical features in people with darker skin tones include:
plaques that may appear more violaceous in color instead of pink or erythematous
higher body surface area of involvement4 and thicker, more scaly plaques6
increased likelihood of postinflammatory hyperpigmentation (PIH).
Worth noting Although individuals of all skin tones may experience the psychosocial impact of psoriasis, quality-of-life measures have been found to be worse in those with skin of color (SOC) compared to White patients.1,4 This may be due to the lingering PIH and hypopigmentation that occurs even after inflammatory plaques are treated. Of course, lack of access to care contributes to greater disease burden and more devastating psychological impact.
Health disparity highlight Psoriasis may be underreported and underdiagnosed in individuals with SOC, as factors contributing to health care disparities may play a role, such as access to health care in general,1,7 and access to clinicians proficient in diagnosing cutaneous diseases in SOC may be delayed.8
Biologic medications are used less often in Black patients than in White patients, despite biologic medications being very efficacious for treatment of psoriasis.1,9,10
Photographs courtesy of Richard P. Usatine, MD.
The Comparison
A Elbow and forearm with erythematous, well-demarcated, pink plaques with mild micaceous scale in a 42-year-old White woman.
B Elbow and forearm with violaceous, well-demarcated plaques with micaceous scale and hyperpigmented patches around the active plaques in a 58-year-old Black man.
Epidemiology Psoriasis prevalence in the United States has been estimated at 3.7%.1-3 If broken down by race or ethnicity, the prevalence of psoriasis varies: 2.5% to 3.7% in White adults1-4; 1.3% to 2% in Black adults1-4; 1.6% in Hispanics/ other adults1-3; 1% in children overall; 0.29% in White children1,5; and 0.06% in Black children.1,5
Key clinical features in people with darker skin tones include:
plaques that may appear more violaceous in color instead of pink or erythematous
higher body surface area of involvement4 and thicker, more scaly plaques6
increased likelihood of postinflammatory hyperpigmentation (PIH).
Worth noting Although individuals of all skin tones may experience the psychosocial impact of psoriasis, quality-of-life measures have been found to be worse in those with skin of color (SOC) compared to White patients.1,4 This may be due to the lingering PIH and hypopigmentation that occurs even after inflammatory plaques are treated. Of course, lack of access to care contributes to greater disease burden and more devastating psychological impact.
Health disparity highlight Psoriasis may be underreported and underdiagnosed in individuals with SOC, as factors contributing to health care disparities may play a role, such as access to health care in general,1,7 and access to clinicians proficient in diagnosing cutaneous diseases in SOC may be delayed.8
Biologic medications are used less often in Black patients than in White patients, despite biologic medications being very efficacious for treatment of psoriasis.1,9,10
References
Kaufman BP, Alexis AF. Psoriasis in skin of color: insights into the epidemiology, clinical presentation, genetics, quality-of-life impact, and treatment of psoriasis in non-white racial/ethnic groups. Am J Clin Dermatol. 2018;19:405-423.
Rachakonda TD, Schupp CW, Armstrong AW. Psoriasis prevalence among adults in the United States. J Am Acad Dermatol. 2014;70:512-516.
Helmick CG, Lee-Han H, Hirsch SC, et al. Prevalence of psoriasis among adults in the U.S.: 2003-2006 and 2009-2010 National Health and Nutrition Examination Surveys. Am J Prev Med. 2014;47:37-45.
Gelfand JM, Stern RS, Nijsten T, et al. The prevalence of psoriasis in African Americans: results from a population-based study. J Am Acad Dermatol. 2005;52:23-26.
Wu JJ, Black MH, Smith N, et al. Low prevalence of psoriasis among children and adolescents in a large multiethnic cohort in southern California. J Am Acad Dermatol. 2011;65:957-964.
Davis SA, Narahari S, Feldman SR, et al. Top dermatologic conditions in patients of color: an analysis of nationally representative data. J Drugs Dermatol. 2012;11:466-473.
Alexis AF, Blackcloud P. Psoriasis in skin of color: epidemiology, genetics, clinical presentation, and treatment nuances. J Clin Aesthet Dermatol. 2014;7:16-24.
Mundluru SN, Ramalingam ND, Tran HN. Addressing internal medicine residents’ discomfort with basic dermatology in persons of color in the primary care clinic. Am J Med Qual. 2019;34:513-513.
Kerr GS, Qaiyumi S, Richards J, et al. Psoriasis and psoriatic arthritis in African-American patients—the need to measure disease burden. Clin Rheumatol. 2015;34:1753-1759.
Takeshita J, Gelfand JM, Li P, et al. Psoriasis in the US Medicare population: prevalence, treatment, and factors associated with biologic use. J Invest Dermatol. 2015;135:2955-2963.
References
Kaufman BP, Alexis AF. Psoriasis in skin of color: insights into the epidemiology, clinical presentation, genetics, quality-of-life impact, and treatment of psoriasis in non-white racial/ethnic groups. Am J Clin Dermatol. 2018;19:405-423.
Rachakonda TD, Schupp CW, Armstrong AW. Psoriasis prevalence among adults in the United States. J Am Acad Dermatol. 2014;70:512-516.
Helmick CG, Lee-Han H, Hirsch SC, et al. Prevalence of psoriasis among adults in the U.S.: 2003-2006 and 2009-2010 National Health and Nutrition Examination Surveys. Am J Prev Med. 2014;47:37-45.
Gelfand JM, Stern RS, Nijsten T, et al. The prevalence of psoriasis in African Americans: results from a population-based study. J Am Acad Dermatol. 2005;52:23-26.
Wu JJ, Black MH, Smith N, et al. Low prevalence of psoriasis among children and adolescents in a large multiethnic cohort in southern California. J Am Acad Dermatol. 2011;65:957-964.
Davis SA, Narahari S, Feldman SR, et al. Top dermatologic conditions in patients of color: an analysis of nationally representative data. J Drugs Dermatol. 2012;11:466-473.
Alexis AF, Blackcloud P. Psoriasis in skin of color: epidemiology, genetics, clinical presentation, and treatment nuances. J Clin Aesthet Dermatol. 2014;7:16-24.
Mundluru SN, Ramalingam ND, Tran HN. Addressing internal medicine residents’ discomfort with basic dermatology in persons of color in the primary care clinic. Am J Med Qual. 2019;34:513-513.
Kerr GS, Qaiyumi S, Richards J, et al. Psoriasis and psoriatic arthritis in African-American patients—the need to measure disease burden. Clin Rheumatol. 2015;34:1753-1759.
Takeshita J, Gelfand JM, Li P, et al. Psoriasis in the US Medicare population: prevalence, treatment, and factors associated with biologic use. J Invest Dermatol. 2015;135:2955-2963.
Panfolliculoma is a rare tumor of follicular origin.1 Clinical examination can reveal a papule, nodule, or tumor that typically is mistaken for an epidermal inclusion cyst, trichoepithelioma, or basal cell carcinoma (BCC).2 As with other benign follicular neoplasms, it often exhibits a protracted growth pattern.3,4 Most cases reported in the literature have been shown to occur in the head or neck region. One hypothesis is that separation into the various components of the hair follicle occurs at a higher frequency in areas with a higher hair density such as the face and scalp.4 The lesion typically presents in patients aged 20 to 70 years, as in our patient, with cases equally distributed among males and females.4,5 Neill et al1 reported a rare case of cystic panfolliculoma occurring on the right forearm of a 64-year-old woman.
As its name suggests, panfolliculoma is exceptional in that it displays features of all segments of the hair follicle, including the infundibulum, isthmus, stem, and bulb.6 Although not necessary for diagnosis, immunohistochemical staining can be utilized to identify each hair follicle component on histopathologic examination. Panfolliculoma stains positive for 34βE12 and cytokeratin 5/6, highlighting infundibular and isthmus keratinocytes and the outer root sheath, respectively. Additionally, Ber-EP4 labels germinative cells, while CD34 highlights contiguous fibrotic stroma and trichilemmal areas.3,4
In our patient, histopathology revealed a cystic structure that was lined by an infundibular epithelium with a prominent granular layer. Solid collections of basaloid germinative cells that demonstrated peripheral palisading were observed (quiz image [top]). Cells with trichohyalin granules, indicative of inner root sheath differentiation, were encased by matrical cells (quiz image [bottom]).
Historically, panfolliculomas characteristically have been known to reside in the dermis, with only focal connection to the epidermis, if at all present. Nevertheless, Harris et al7 detailed 2 cases that displayed predominant epidermal involvement, defined by the term epidermal panfolliculoma. In a study performed by Shan and Guo,2 an additional 9 cases (19 panfolliculomas) were found to have similar findings, for which the term superficial panfolliculoma was suggested. In cases that display a primary epidermal component, common mimickers include tumor of the follicular infundibulum and the reactive process of follicular induction.7
Cystic panfolliculoma is a rare subtype further characterized as a lesion with distinctive features of a panfolliculoma that arises from a cyst wall composed of the follicular infundibulum.2,6 The origin of cystic panfolliculoma has not been fully elucidated. It has been hypothesized that the formation of such lesions may arise due to epithelial-mesenchymal interactions. One explanation is that basal cells with stem cell capability may progress into hair follicle structures after communication with underlying dermal cells during invagination of the epidermis, while the epithelial cells not in close proximity to dermal cells maintain stem cell capability.8
The histologic differential diagnosis of cystic panfolliculoma includes dilated pore of Winer, epidermal inclusion cyst, pilar cyst, trichofolliculoma, folliculosebaceous cystic hamartoma, cystic trichoblastoma, and BCC.5 Panfolliculoma can mimic both trichoblastoma and trichoepithelioma on a low-power field; however, the latter follicular tumors lack differentiation to the infundibulum, isthmus, outer root sheath, or hair shaft, as in a panfolliculoma.4 Trichoblastoma is composed of germinative hair follicle cells, with differentiation limited to the hair germ and papilla (Figure 1).9 Panfolliculoma additionally differs from trichoblastoma by having a more prevalent epithelial factor compared to a more pronounced stromal factor in trichoblastoma.1 The cystic subtype of trichoblastoma differs from cystic panfolliculoma in that the cyst wall develops from the infundibulum only and has germinative cells protruding outwards from the cyst wall.
Figure 1. Trichoblastoma. Basaloid islands composed of germinative hair follicle cells with differentiation limited to the hair germ and papilla with concentric fibroblast-rich stroma and lack of retraction (H&E, original magnification ×40).
Although BCCs may arise in cystic structures, panfolliculomas can be discerned from this entity by their sharp demarcation, lack of peritumoral clefting, and presence of cytokeratin 20-positive Merkel cells.5 Unlike panfolliculoma, the tumor islands in BCC commonly display peripheral palisading of nuclei with a surrounding fibromyxoid stroma (Figure 2). Additionally, BCCs can exhibit crowding of nuclei, atypia, and mitoses.6
Figure 2. Basal cell carcinoma. Basaloid islands with peripheral palisading of nuclei, retraction artifact, and fibromyxoid stroma (H&E, original magnification ×40).
Folliculosebaceous cystic hamartomas and cystic panfolliculomas both contain a cystic structure with differentiation of the cyst wall to the hair follicle. However, folliculosebaceous cystic hamartomas are dilated infundibulocystic configurations that contain sebaceous glands emanating from the cyst wall (Figure 3). Kimura et al10 described defining features of the mesenchymal component of this follicular tumor, including an increase in fibroplasia, vascularity, and adipose tissue. In addition, the epithelial aspect exhibits clefting among the stroma and uninvolved dermis.6
Figure 3. Folliculosebaceous cystic hamartoma. Dilated infundibulocystic structure with sebaceous glands emanating from the cyst wall (H&E, original magnification ×40).
Dilated pore of Winer consists of a cystic opening with connection to the epidermis. The cyst wall resembles the follicular infundibulum, and the cavity is filled with lamellar orthokeratosis (Figure 4).5,11 Epidermal inclusion cysts also contain a cyst wall that resembles the infundibular epithelium, without differentiation to all segments of the hair follicle. They are lined by a stratified squamous epithelium, retain a granular layer, and contain lamellar keratin within the cyst cavity.5,12
Figure 4. Dilated pore of Winer. Dilated follicular infundibulum with radiating epithelial protrusions and central keratinous material (H&E, original magnification ×40).
In summary, panfolliculoma is a rare benign neoplasm that demonstrates differentiation to each component of the hair follicle structure. Our case demonstrates a unique subtype showcasing cystic changes that infrequently has been described in the literature.
References
Neill B, Bingham C, Braudis K, et al. A rare cutaneous adnexal neoplasm: cystic panfolliculoma. J Cutan Pathol. 2016;43:1183-1185.
Shan SJ, Guo Y. Panfolliculoma and histopathologic variants: a study of 19 cases. Am J Dermatopathol. 2014;36:965-971.
Huang CY, Wu YH. Panfolliculoma: report of two cases. Dermatol Sínica. 2010;28:73-76.
Alkhalidi HM, Alhumaidy AA. Cystic panfolliculoma of the scalp: report of a very rare case and brief review. Indian J Pathol Microbiol. 2013;56:437-439.
López-Takegami JC, Wolter M, Löser C, et al. Classification of cysts with follicular germinative differentiation. J Cutan Pathol. 2016;43:191-199.
Harris A, Faulkner-Jones B, Zimarowski MJ. Epidermal panfolliculoma: a report of 2 cases. Am J Dermatopathol. 2011;33:E7-E10.
Fukuyama M, Sato Y, Yamazaki Y, et al. Immunohistochemical dissection of cystic panfolliculoma focusing on the expression of multiple hair follicle lineage markers with an insight into the pathogenesis. J Cutan Pathol. 2017;44:861-866.
Tellechea O, Cardoso JC, Reis JP, et al. Benign follicular tumors. An Bras Dermatol. 2015;90:780-796; quiz 797-788.
Kimura T, Miyazawa H, Aoyagi T, et al. Folliculosebaceous cystic hamartoma. a distinctive malformation of the skin. Am J Dermatopathol. 1991;13:213-220.
Misago N, Inoue T, Narisawa Y. Cystic trichoblastoma: a report of two cases with an immunohistochemical study. J Dermatol. 2015;42:305-310.
From the Department of Dermatology, University of Florida College of Medicine, Gainesville.
The authors report no conflict of interest.
Correspondence: Elizabeth L. Bisbee, MD, Department of Dermatology, University of Florida College of Medicine, 4037 NW 86th Terrace, 4th Floor, Gainesville, FL 32606 (ebisbee@dermatology.med.ufl.edu).
From the Department of Dermatology, University of Florida College of Medicine, Gainesville.
The authors report no conflict of interest.
Correspondence: Elizabeth L. Bisbee, MD, Department of Dermatology, University of Florida College of Medicine, 4037 NW 86th Terrace, 4th Floor, Gainesville, FL 32606 (ebisbee@dermatology.med.ufl.edu).
Author and Disclosure Information
From the Department of Dermatology, University of Florida College of Medicine, Gainesville.
The authors report no conflict of interest.
Correspondence: Elizabeth L. Bisbee, MD, Department of Dermatology, University of Florida College of Medicine, 4037 NW 86th Terrace, 4th Floor, Gainesville, FL 32606 (ebisbee@dermatology.med.ufl.edu).
Panfolliculoma is a rare tumor of follicular origin.1 Clinical examination can reveal a papule, nodule, or tumor that typically is mistaken for an epidermal inclusion cyst, trichoepithelioma, or basal cell carcinoma (BCC).2 As with other benign follicular neoplasms, it often exhibits a protracted growth pattern.3,4 Most cases reported in the literature have been shown to occur in the head or neck region. One hypothesis is that separation into the various components of the hair follicle occurs at a higher frequency in areas with a higher hair density such as the face and scalp.4 The lesion typically presents in patients aged 20 to 70 years, as in our patient, with cases equally distributed among males and females.4,5 Neill et al1 reported a rare case of cystic panfolliculoma occurring on the right forearm of a 64-year-old woman.
As its name suggests, panfolliculoma is exceptional in that it displays features of all segments of the hair follicle, including the infundibulum, isthmus, stem, and bulb.6 Although not necessary for diagnosis, immunohistochemical staining can be utilized to identify each hair follicle component on histopathologic examination. Panfolliculoma stains positive for 34βE12 and cytokeratin 5/6, highlighting infundibular and isthmus keratinocytes and the outer root sheath, respectively. Additionally, Ber-EP4 labels germinative cells, while CD34 highlights contiguous fibrotic stroma and trichilemmal areas.3,4
In our patient, histopathology revealed a cystic structure that was lined by an infundibular epithelium with a prominent granular layer. Solid collections of basaloid germinative cells that demonstrated peripheral palisading were observed (quiz image [top]). Cells with trichohyalin granules, indicative of inner root sheath differentiation, were encased by matrical cells (quiz image [bottom]).
Historically, panfolliculomas characteristically have been known to reside in the dermis, with only focal connection to the epidermis, if at all present. Nevertheless, Harris et al7 detailed 2 cases that displayed predominant epidermal involvement, defined by the term epidermal panfolliculoma. In a study performed by Shan and Guo,2 an additional 9 cases (19 panfolliculomas) were found to have similar findings, for which the term superficial panfolliculoma was suggested. In cases that display a primary epidermal component, common mimickers include tumor of the follicular infundibulum and the reactive process of follicular induction.7
Cystic panfolliculoma is a rare subtype further characterized as a lesion with distinctive features of a panfolliculoma that arises from a cyst wall composed of the follicular infundibulum.2,6 The origin of cystic panfolliculoma has not been fully elucidated. It has been hypothesized that the formation of such lesions may arise due to epithelial-mesenchymal interactions. One explanation is that basal cells with stem cell capability may progress into hair follicle structures after communication with underlying dermal cells during invagination of the epidermis, while the epithelial cells not in close proximity to dermal cells maintain stem cell capability.8
The histologic differential diagnosis of cystic panfolliculoma includes dilated pore of Winer, epidermal inclusion cyst, pilar cyst, trichofolliculoma, folliculosebaceous cystic hamartoma, cystic trichoblastoma, and BCC.5 Panfolliculoma can mimic both trichoblastoma and trichoepithelioma on a low-power field; however, the latter follicular tumors lack differentiation to the infundibulum, isthmus, outer root sheath, or hair shaft, as in a panfolliculoma.4 Trichoblastoma is composed of germinative hair follicle cells, with differentiation limited to the hair germ and papilla (Figure 1).9 Panfolliculoma additionally differs from trichoblastoma by having a more prevalent epithelial factor compared to a more pronounced stromal factor in trichoblastoma.1 The cystic subtype of trichoblastoma differs from cystic panfolliculoma in that the cyst wall develops from the infundibulum only and has germinative cells protruding outwards from the cyst wall.
Figure 1. Trichoblastoma. Basaloid islands composed of germinative hair follicle cells with differentiation limited to the hair germ and papilla with concentric fibroblast-rich stroma and lack of retraction (H&E, original magnification ×40).
Although BCCs may arise in cystic structures, panfolliculomas can be discerned from this entity by their sharp demarcation, lack of peritumoral clefting, and presence of cytokeratin 20-positive Merkel cells.5 Unlike panfolliculoma, the tumor islands in BCC commonly display peripheral palisading of nuclei with a surrounding fibromyxoid stroma (Figure 2). Additionally, BCCs can exhibit crowding of nuclei, atypia, and mitoses.6
Figure 2. Basal cell carcinoma. Basaloid islands with peripheral palisading of nuclei, retraction artifact, and fibromyxoid stroma (H&E, original magnification ×40).
Folliculosebaceous cystic hamartomas and cystic panfolliculomas both contain a cystic structure with differentiation of the cyst wall to the hair follicle. However, folliculosebaceous cystic hamartomas are dilated infundibulocystic configurations that contain sebaceous glands emanating from the cyst wall (Figure 3). Kimura et al10 described defining features of the mesenchymal component of this follicular tumor, including an increase in fibroplasia, vascularity, and adipose tissue. In addition, the epithelial aspect exhibits clefting among the stroma and uninvolved dermis.6
Figure 3. Folliculosebaceous cystic hamartoma. Dilated infundibulocystic structure with sebaceous glands emanating from the cyst wall (H&E, original magnification ×40).
Dilated pore of Winer consists of a cystic opening with connection to the epidermis. The cyst wall resembles the follicular infundibulum, and the cavity is filled with lamellar orthokeratosis (Figure 4).5,11 Epidermal inclusion cysts also contain a cyst wall that resembles the infundibular epithelium, without differentiation to all segments of the hair follicle. They are lined by a stratified squamous epithelium, retain a granular layer, and contain lamellar keratin within the cyst cavity.5,12
Figure 4. Dilated pore of Winer. Dilated follicular infundibulum with radiating epithelial protrusions and central keratinous material (H&E, original magnification ×40).
In summary, panfolliculoma is a rare benign neoplasm that demonstrates differentiation to each component of the hair follicle structure. Our case demonstrates a unique subtype showcasing cystic changes that infrequently has been described in the literature.
The Diagnosis: Cystic Panfolliculoma
Panfolliculoma is a rare tumor of follicular origin.1 Clinical examination can reveal a papule, nodule, or tumor that typically is mistaken for an epidermal inclusion cyst, trichoepithelioma, or basal cell carcinoma (BCC).2 As with other benign follicular neoplasms, it often exhibits a protracted growth pattern.3,4 Most cases reported in the literature have been shown to occur in the head or neck region. One hypothesis is that separation into the various components of the hair follicle occurs at a higher frequency in areas with a higher hair density such as the face and scalp.4 The lesion typically presents in patients aged 20 to 70 years, as in our patient, with cases equally distributed among males and females.4,5 Neill et al1 reported a rare case of cystic panfolliculoma occurring on the right forearm of a 64-year-old woman.
As its name suggests, panfolliculoma is exceptional in that it displays features of all segments of the hair follicle, including the infundibulum, isthmus, stem, and bulb.6 Although not necessary for diagnosis, immunohistochemical staining can be utilized to identify each hair follicle component on histopathologic examination. Panfolliculoma stains positive for 34βE12 and cytokeratin 5/6, highlighting infundibular and isthmus keratinocytes and the outer root sheath, respectively. Additionally, Ber-EP4 labels germinative cells, while CD34 highlights contiguous fibrotic stroma and trichilemmal areas.3,4
In our patient, histopathology revealed a cystic structure that was lined by an infundibular epithelium with a prominent granular layer. Solid collections of basaloid germinative cells that demonstrated peripheral palisading were observed (quiz image [top]). Cells with trichohyalin granules, indicative of inner root sheath differentiation, were encased by matrical cells (quiz image [bottom]).
Historically, panfolliculomas characteristically have been known to reside in the dermis, with only focal connection to the epidermis, if at all present. Nevertheless, Harris et al7 detailed 2 cases that displayed predominant epidermal involvement, defined by the term epidermal panfolliculoma. In a study performed by Shan and Guo,2 an additional 9 cases (19 panfolliculomas) were found to have similar findings, for which the term superficial panfolliculoma was suggested. In cases that display a primary epidermal component, common mimickers include tumor of the follicular infundibulum and the reactive process of follicular induction.7
Cystic panfolliculoma is a rare subtype further characterized as a lesion with distinctive features of a panfolliculoma that arises from a cyst wall composed of the follicular infundibulum.2,6 The origin of cystic panfolliculoma has not been fully elucidated. It has been hypothesized that the formation of such lesions may arise due to epithelial-mesenchymal interactions. One explanation is that basal cells with stem cell capability may progress into hair follicle structures after communication with underlying dermal cells during invagination of the epidermis, while the epithelial cells not in close proximity to dermal cells maintain stem cell capability.8
The histologic differential diagnosis of cystic panfolliculoma includes dilated pore of Winer, epidermal inclusion cyst, pilar cyst, trichofolliculoma, folliculosebaceous cystic hamartoma, cystic trichoblastoma, and BCC.5 Panfolliculoma can mimic both trichoblastoma and trichoepithelioma on a low-power field; however, the latter follicular tumors lack differentiation to the infundibulum, isthmus, outer root sheath, or hair shaft, as in a panfolliculoma.4 Trichoblastoma is composed of germinative hair follicle cells, with differentiation limited to the hair germ and papilla (Figure 1).9 Panfolliculoma additionally differs from trichoblastoma by having a more prevalent epithelial factor compared to a more pronounced stromal factor in trichoblastoma.1 The cystic subtype of trichoblastoma differs from cystic panfolliculoma in that the cyst wall develops from the infundibulum only and has germinative cells protruding outwards from the cyst wall.
Figure 1. Trichoblastoma. Basaloid islands composed of germinative hair follicle cells with differentiation limited to the hair germ and papilla with concentric fibroblast-rich stroma and lack of retraction (H&E, original magnification ×40).
Although BCCs may arise in cystic structures, panfolliculomas can be discerned from this entity by their sharp demarcation, lack of peritumoral clefting, and presence of cytokeratin 20-positive Merkel cells.5 Unlike panfolliculoma, the tumor islands in BCC commonly display peripheral palisading of nuclei with a surrounding fibromyxoid stroma (Figure 2). Additionally, BCCs can exhibit crowding of nuclei, atypia, and mitoses.6
Figure 2. Basal cell carcinoma. Basaloid islands with peripheral palisading of nuclei, retraction artifact, and fibromyxoid stroma (H&E, original magnification ×40).
Folliculosebaceous cystic hamartomas and cystic panfolliculomas both contain a cystic structure with differentiation of the cyst wall to the hair follicle. However, folliculosebaceous cystic hamartomas are dilated infundibulocystic configurations that contain sebaceous glands emanating from the cyst wall (Figure 3). Kimura et al10 described defining features of the mesenchymal component of this follicular tumor, including an increase in fibroplasia, vascularity, and adipose tissue. In addition, the epithelial aspect exhibits clefting among the stroma and uninvolved dermis.6
Figure 3. Folliculosebaceous cystic hamartoma. Dilated infundibulocystic structure with sebaceous glands emanating from the cyst wall (H&E, original magnification ×40).
Dilated pore of Winer consists of a cystic opening with connection to the epidermis. The cyst wall resembles the follicular infundibulum, and the cavity is filled with lamellar orthokeratosis (Figure 4).5,11 Epidermal inclusion cysts also contain a cyst wall that resembles the infundibular epithelium, without differentiation to all segments of the hair follicle. They are lined by a stratified squamous epithelium, retain a granular layer, and contain lamellar keratin within the cyst cavity.5,12
Figure 4. Dilated pore of Winer. Dilated follicular infundibulum with radiating epithelial protrusions and central keratinous material (H&E, original magnification ×40).
In summary, panfolliculoma is a rare benign neoplasm that demonstrates differentiation to each component of the hair follicle structure. Our case demonstrates a unique subtype showcasing cystic changes that infrequently has been described in the literature.
References
Neill B, Bingham C, Braudis K, et al. A rare cutaneous adnexal neoplasm: cystic panfolliculoma. J Cutan Pathol. 2016;43:1183-1185.
Shan SJ, Guo Y. Panfolliculoma and histopathologic variants: a study of 19 cases. Am J Dermatopathol. 2014;36:965-971.
Huang CY, Wu YH. Panfolliculoma: report of two cases. Dermatol Sínica. 2010;28:73-76.
Alkhalidi HM, Alhumaidy AA. Cystic panfolliculoma of the scalp: report of a very rare case and brief review. Indian J Pathol Microbiol. 2013;56:437-439.
López-Takegami JC, Wolter M, Löser C, et al. Classification of cysts with follicular germinative differentiation. J Cutan Pathol. 2016;43:191-199.
Harris A, Faulkner-Jones B, Zimarowski MJ. Epidermal panfolliculoma: a report of 2 cases. Am J Dermatopathol. 2011;33:E7-E10.
Fukuyama M, Sato Y, Yamazaki Y, et al. Immunohistochemical dissection of cystic panfolliculoma focusing on the expression of multiple hair follicle lineage markers with an insight into the pathogenesis. J Cutan Pathol. 2017;44:861-866.
Tellechea O, Cardoso JC, Reis JP, et al. Benign follicular tumors. An Bras Dermatol. 2015;90:780-796; quiz 797-788.
Kimura T, Miyazawa H, Aoyagi T, et al. Folliculosebaceous cystic hamartoma. a distinctive malformation of the skin. Am J Dermatopathol. 1991;13:213-220.
Misago N, Inoue T, Narisawa Y. Cystic trichoblastoma: a report of two cases with an immunohistochemical study. J Dermatol. 2015;42:305-310.
Huang CY, Wu YH. Panfolliculoma: report of two cases. Dermatol Sínica. 2010;28:73-76.
Alkhalidi HM, Alhumaidy AA. Cystic panfolliculoma of the scalp: report of a very rare case and brief review. Indian J Pathol Microbiol. 2013;56:437-439.
López-Takegami JC, Wolter M, Löser C, et al. Classification of cysts with follicular germinative differentiation. J Cutan Pathol. 2016;43:191-199.
Harris A, Faulkner-Jones B, Zimarowski MJ. Epidermal panfolliculoma: a report of 2 cases. Am J Dermatopathol. 2011;33:E7-E10.
Fukuyama M, Sato Y, Yamazaki Y, et al. Immunohistochemical dissection of cystic panfolliculoma focusing on the expression of multiple hair follicle lineage markers with an insight into the pathogenesis. J Cutan Pathol. 2017;44:861-866.
Tellechea O, Cardoso JC, Reis JP, et al. Benign follicular tumors. An Bras Dermatol. 2015;90:780-796; quiz 797-788.
Kimura T, Miyazawa H, Aoyagi T, et al. Folliculosebaceous cystic hamartoma. a distinctive malformation of the skin. Am J Dermatopathol. 1991;13:213-220.
Misago N, Inoue T, Narisawa Y. Cystic trichoblastoma: a report of two cases with an immunohistochemical study. J Dermatol. 2015;42:305-310.
A healthy 45-year-old man presented to the dermatology clinic with a slow-growing subcutaneous nodule on the left chest that had been present for years.
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Biopsy revealed a cellular neoplasm consisting of atypical polygonal cells with a hobnailed appearance, vasoformative characteristics, and rare extravasated erythrocytes. The tumor had an infiltrative growth pattern as demonstrated by dissecting dermal collagen and a poorly defined border with adjacent normal tissue (Figure 1). Immunohistochemistry revealed that the lesion was positive for CD31 and D2-40 (Figure 2) but negative for cytokeratin, CD10, CD68, human herpesvirus 8, CD34, and Melan A, thus confirming the endothelial origin of the tumor cells and the diagnosis of cutaneous angiosarcoma (CAS). The patient was treated with extended surgical excision and radiation therapy. No recurrence or metastasis was found throughout 2 years of follow-up.
Figure 1. A and B, Histologic examination revealed a cellular neoplasm consisting of atypical polygonal cells forming irregular channels and dissecting dermal collagen (H&E, original magnifications ×40 and ×200).
Figure 2. A and B, The endothelial origin was confirmed by immunohistochemistry for CD31 and D2-40, respectively (original magnifications ×100 and ×100). Angiosarcoma is a highly aggressive malignant neoplasm derived from vascular endothelial cells, most commonly involving the skin and superficial soft tissue. Angiosarcoma can be subdivided into CAS and visceral angiosarcoma according to the primary site of the tumor.1 Accurate and timely diagnosis of CAS is paramount due to its poor prognostic outcomes despite aggressive treatments. Clinically, CAS most frequently presents asymptomatically as an enlarging purple-red or bruiselike lesion with poorly defined margins. Cutaneous angiosarcoma often is misdiagnosed as an ecchymosis or hematoma due to its initial subtle presentation. It also may resemble eczema, hemangioma, and cellulitis; advanced lesions can mimic epithelial or mesenchymal neoplasms, including squamous cell carcinoma, keratoacanthoma, basal cell carcinoma, atypical fibroxanthoma (AFX), and malignant melanoma.2 Our patient lacked the classic clinical presentation of a hematomalike lesion and characteristic histologic features of anastomosing vascular structures with abundant extravasated erythrocytes at low magnification. However, the presence of erythrocytes in vascular channels along with CD31 and D2-40 immunoreactivity confirmed its vascular origin. The prognosis of CAS is poor even with localized lesions. Age is a substantial prognostic factor, as a near 50% reduction of overall survival rate has been observed in patients older than 50 years.3 Other reported poor predictors for prognosis include male sex, the presence of cardiovascular diseases, location on the scalp, history of smoking, tumor size larger than 5 cm, and the presence of satellite lesions. Distant metastases are common, primarily affecting the lungs but also the bones and liver.4
Radical resection with a negative margin is considered the first-line treatment of choice. Although there is a paucity of studies assessing the specific width of surgical margins, application of no less than a 3-cm peripheral margin as well as a clear deep margin is recommended.5 Adjuvant radiation therapy also is essential to prevent local recurrence. Patients receiving combination therapy have a superior overall survival rate when compared to those undergoing surgery or radiation therapy alone.4
Cutaneous follicle center lymphoma also may present as 1 or more localized erythematous papules, plaques, and/or nodules, commonly arising on the scalp/forehead or trunk of middle-aged men. Despite being a low-grade lymphoma with a favorable prognosis, it may have a relatively fast growth and locally aggressive course if left untreated. The distinguishing histologic feature is a dense proliferation of neoplastic infiltrates in the dermis, which is separated from the epidermis by the grenz zone.6
The clinical presentation of cutaneous metastatic carcinomas varies greatly, with 1 or multiple localized or widespread lesions commonly involving the abdominal wall, scalp, and face. The lesions also may mimic benign dermatologic conditions, thus potentially resulting in erroneous clinical diagnosis and delayed therapy of the primary malignancy. Obtaining clinical history is crucial; however, a precise diagnosis may require histologic examination.7
Atypical fibroxanthoma is a rare superficial cutaneous sarcoma that typically occurs on the head and neck in sun-damaged elderly individuals. Clinically, AFX presents as well-circumscribed red or pink nodules or plaques with or without ulceration, crust, or scale.8 Atypical fibroxanthoma lesions usually are small, with a median diameter of 1 cm, while those greater than 2 cm reportedly account for less than 5% of cases.9 Atypical fibroxanthoma typically grows rapidly with no pain or discomfort. Histologically, AFX is characterized by a well-circumscribed dermal nodule consisting of pleomorphic spindle cells and multinucleated giant cells that can stain positively for CD10 and procollagen 1.10
Cutaneous pseudolymphoma is a benign inflammatory response process that stimulates polyclonal T- or B-cell lymphoproliferation. The clinical presentation may appear as localized or disseminated flesh-colored or red papules, infiltrated plaques, and nodules.11 Histopathology will show mixtures of B and T cells along with dendritic cells and macrophages, but irregular vascular structure and dissecting dermal collagen are not involved.
We present an unusual case of CAS with multiple pink nodules on the scalp. Early biopsy of these lesions is important to reach a correct diagnosis and to initiate appropriate treatment.
References
Ishida Y, Otsuka A, Kabashima K. Cutaneous angiosarcoma: update on biology and latest treatment. Curr Opin Oncol. 2018;30:107-112.
Dossett LA, Harrington M, Cruse CW, et al. Cutaneous angiosarcoma. Curr Probl Cancer. 2015;39:258-263.
Albores-Saavedra J, Schwartz AM, Henson DE, et al. Cutaneous angiosarcoma. analysis of 434 cases from the surveillance, epidemiology, and end results program, 1973-2007. Ann Diagn Pathol. 2011;15:93-97.
Guadagnolo BA, Zagars GK, Araujo D, et al. Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp. Head Neck. 2011;33:661-667.
Lindford A, Böhling T, Vaalavirta L, et al. Surgical management of radiation-associated cutaneous breast angiosarcoma. J Plast Reconstr Aesthet Surg. 2011;64:1036-1042.
Costa EPW, Lu.0cena BD, Amin GA, et al. Primary cutaneous follicle center lymphoma. An Bras Dermatol. 2017;92:701-703.
Menon AR, Thomas AS, Suresh N, et al. Cutaneous metastasis: an unusual presenting feature of urologic malignancies. Urol Ann. 2016;8:377-380.
Iorizzo LJ 3rd, Brown MD. Atypical fibroxanthoma: a review of the literature. Dermatol Surg. 2011;37:146-157.
From the Department of Dermatology and Key Laboratory of Immunodermatology, First Hospital of China Medical University, Shenyan.
The authors report no conflict of interest.
This work was supported by grants from the National Natural Science Foundation of China (81803148) and the National Key Research and Development Program of China (2016YFC0901504).
Correspondence: Song Zheng, MD, First Hospital of China Medical University, Nanjing N St, Heping District, Shenyang 110001, China (zhengsongcmu@163.com).
From the Department of Dermatology and Key Laboratory of Immunodermatology, First Hospital of China Medical University, Shenyan.
The authors report no conflict of interest.
This work was supported by grants from the National Natural Science Foundation of China (81803148) and the National Key Research and Development Program of China (2016YFC0901504).
Correspondence: Song Zheng, MD, First Hospital of China Medical University, Nanjing N St, Heping District, Shenyang 110001, China (zhengsongcmu@163.com).
Author and Disclosure Information
From the Department of Dermatology and Key Laboratory of Immunodermatology, First Hospital of China Medical University, Shenyan.
The authors report no conflict of interest.
This work was supported by grants from the National Natural Science Foundation of China (81803148) and the National Key Research and Development Program of China (2016YFC0901504).
Correspondence: Song Zheng, MD, First Hospital of China Medical University, Nanjing N St, Heping District, Shenyang 110001, China (zhengsongcmu@163.com).
Biopsy revealed a cellular neoplasm consisting of atypical polygonal cells with a hobnailed appearance, vasoformative characteristics, and rare extravasated erythrocytes. The tumor had an infiltrative growth pattern as demonstrated by dissecting dermal collagen and a poorly defined border with adjacent normal tissue (Figure 1). Immunohistochemistry revealed that the lesion was positive for CD31 and D2-40 (Figure 2) but negative for cytokeratin, CD10, CD68, human herpesvirus 8, CD34, and Melan A, thus confirming the endothelial origin of the tumor cells and the diagnosis of cutaneous angiosarcoma (CAS). The patient was treated with extended surgical excision and radiation therapy. No recurrence or metastasis was found throughout 2 years of follow-up.
Figure 1. A and B, Histologic examination revealed a cellular neoplasm consisting of atypical polygonal cells forming irregular channels and dissecting dermal collagen (H&E, original magnifications ×40 and ×200).
Figure 2. A and B, The endothelial origin was confirmed by immunohistochemistry for CD31 and D2-40, respectively (original magnifications ×100 and ×100). Angiosarcoma is a highly aggressive malignant neoplasm derived from vascular endothelial cells, most commonly involving the skin and superficial soft tissue. Angiosarcoma can be subdivided into CAS and visceral angiosarcoma according to the primary site of the tumor.1 Accurate and timely diagnosis of CAS is paramount due to its poor prognostic outcomes despite aggressive treatments. Clinically, CAS most frequently presents asymptomatically as an enlarging purple-red or bruiselike lesion with poorly defined margins. Cutaneous angiosarcoma often is misdiagnosed as an ecchymosis or hematoma due to its initial subtle presentation. It also may resemble eczema, hemangioma, and cellulitis; advanced lesions can mimic epithelial or mesenchymal neoplasms, including squamous cell carcinoma, keratoacanthoma, basal cell carcinoma, atypical fibroxanthoma (AFX), and malignant melanoma.2 Our patient lacked the classic clinical presentation of a hematomalike lesion and characteristic histologic features of anastomosing vascular structures with abundant extravasated erythrocytes at low magnification. However, the presence of erythrocytes in vascular channels along with CD31 and D2-40 immunoreactivity confirmed its vascular origin. The prognosis of CAS is poor even with localized lesions. Age is a substantial prognostic factor, as a near 50% reduction of overall survival rate has been observed in patients older than 50 years.3 Other reported poor predictors for prognosis include male sex, the presence of cardiovascular diseases, location on the scalp, history of smoking, tumor size larger than 5 cm, and the presence of satellite lesions. Distant metastases are common, primarily affecting the lungs but also the bones and liver.4
Radical resection with a negative margin is considered the first-line treatment of choice. Although there is a paucity of studies assessing the specific width of surgical margins, application of no less than a 3-cm peripheral margin as well as a clear deep margin is recommended.5 Adjuvant radiation therapy also is essential to prevent local recurrence. Patients receiving combination therapy have a superior overall survival rate when compared to those undergoing surgery or radiation therapy alone.4
Cutaneous follicle center lymphoma also may present as 1 or more localized erythematous papules, plaques, and/or nodules, commonly arising on the scalp/forehead or trunk of middle-aged men. Despite being a low-grade lymphoma with a favorable prognosis, it may have a relatively fast growth and locally aggressive course if left untreated. The distinguishing histologic feature is a dense proliferation of neoplastic infiltrates in the dermis, which is separated from the epidermis by the grenz zone.6
The clinical presentation of cutaneous metastatic carcinomas varies greatly, with 1 or multiple localized or widespread lesions commonly involving the abdominal wall, scalp, and face. The lesions also may mimic benign dermatologic conditions, thus potentially resulting in erroneous clinical diagnosis and delayed therapy of the primary malignancy. Obtaining clinical history is crucial; however, a precise diagnosis may require histologic examination.7
Atypical fibroxanthoma is a rare superficial cutaneous sarcoma that typically occurs on the head and neck in sun-damaged elderly individuals. Clinically, AFX presents as well-circumscribed red or pink nodules or plaques with or without ulceration, crust, or scale.8 Atypical fibroxanthoma lesions usually are small, with a median diameter of 1 cm, while those greater than 2 cm reportedly account for less than 5% of cases.9 Atypical fibroxanthoma typically grows rapidly with no pain or discomfort. Histologically, AFX is characterized by a well-circumscribed dermal nodule consisting of pleomorphic spindle cells and multinucleated giant cells that can stain positively for CD10 and procollagen 1.10
Cutaneous pseudolymphoma is a benign inflammatory response process that stimulates polyclonal T- or B-cell lymphoproliferation. The clinical presentation may appear as localized or disseminated flesh-colored or red papules, infiltrated plaques, and nodules.11 Histopathology will show mixtures of B and T cells along with dendritic cells and macrophages, but irregular vascular structure and dissecting dermal collagen are not involved.
We present an unusual case of CAS with multiple pink nodules on the scalp. Early biopsy of these lesions is important to reach a correct diagnosis and to initiate appropriate treatment.
The Diagnosis: Cutaneous Angiosarcoma
Biopsy revealed a cellular neoplasm consisting of atypical polygonal cells with a hobnailed appearance, vasoformative characteristics, and rare extravasated erythrocytes. The tumor had an infiltrative growth pattern as demonstrated by dissecting dermal collagen and a poorly defined border with adjacent normal tissue (Figure 1). Immunohistochemistry revealed that the lesion was positive for CD31 and D2-40 (Figure 2) but negative for cytokeratin, CD10, CD68, human herpesvirus 8, CD34, and Melan A, thus confirming the endothelial origin of the tumor cells and the diagnosis of cutaneous angiosarcoma (CAS). The patient was treated with extended surgical excision and radiation therapy. No recurrence or metastasis was found throughout 2 years of follow-up.
Figure 1. A and B, Histologic examination revealed a cellular neoplasm consisting of atypical polygonal cells forming irregular channels and dissecting dermal collagen (H&E, original magnifications ×40 and ×200).
Figure 2. A and B, The endothelial origin was confirmed by immunohistochemistry for CD31 and D2-40, respectively (original magnifications ×100 and ×100). Angiosarcoma is a highly aggressive malignant neoplasm derived from vascular endothelial cells, most commonly involving the skin and superficial soft tissue. Angiosarcoma can be subdivided into CAS and visceral angiosarcoma according to the primary site of the tumor.1 Accurate and timely diagnosis of CAS is paramount due to its poor prognostic outcomes despite aggressive treatments. Clinically, CAS most frequently presents asymptomatically as an enlarging purple-red or bruiselike lesion with poorly defined margins. Cutaneous angiosarcoma often is misdiagnosed as an ecchymosis or hematoma due to its initial subtle presentation. It also may resemble eczema, hemangioma, and cellulitis; advanced lesions can mimic epithelial or mesenchymal neoplasms, including squamous cell carcinoma, keratoacanthoma, basal cell carcinoma, atypical fibroxanthoma (AFX), and malignant melanoma.2 Our patient lacked the classic clinical presentation of a hematomalike lesion and characteristic histologic features of anastomosing vascular structures with abundant extravasated erythrocytes at low magnification. However, the presence of erythrocytes in vascular channels along with CD31 and D2-40 immunoreactivity confirmed its vascular origin. The prognosis of CAS is poor even with localized lesions. Age is a substantial prognostic factor, as a near 50% reduction of overall survival rate has been observed in patients older than 50 years.3 Other reported poor predictors for prognosis include male sex, the presence of cardiovascular diseases, location on the scalp, history of smoking, tumor size larger than 5 cm, and the presence of satellite lesions. Distant metastases are common, primarily affecting the lungs but also the bones and liver.4
Radical resection with a negative margin is considered the first-line treatment of choice. Although there is a paucity of studies assessing the specific width of surgical margins, application of no less than a 3-cm peripheral margin as well as a clear deep margin is recommended.5 Adjuvant radiation therapy also is essential to prevent local recurrence. Patients receiving combination therapy have a superior overall survival rate when compared to those undergoing surgery or radiation therapy alone.4
Cutaneous follicle center lymphoma also may present as 1 or more localized erythematous papules, plaques, and/or nodules, commonly arising on the scalp/forehead or trunk of middle-aged men. Despite being a low-grade lymphoma with a favorable prognosis, it may have a relatively fast growth and locally aggressive course if left untreated. The distinguishing histologic feature is a dense proliferation of neoplastic infiltrates in the dermis, which is separated from the epidermis by the grenz zone.6
The clinical presentation of cutaneous metastatic carcinomas varies greatly, with 1 or multiple localized or widespread lesions commonly involving the abdominal wall, scalp, and face. The lesions also may mimic benign dermatologic conditions, thus potentially resulting in erroneous clinical diagnosis and delayed therapy of the primary malignancy. Obtaining clinical history is crucial; however, a precise diagnosis may require histologic examination.7
Atypical fibroxanthoma is a rare superficial cutaneous sarcoma that typically occurs on the head and neck in sun-damaged elderly individuals. Clinically, AFX presents as well-circumscribed red or pink nodules or plaques with or without ulceration, crust, or scale.8 Atypical fibroxanthoma lesions usually are small, with a median diameter of 1 cm, while those greater than 2 cm reportedly account for less than 5% of cases.9 Atypical fibroxanthoma typically grows rapidly with no pain or discomfort. Histologically, AFX is characterized by a well-circumscribed dermal nodule consisting of pleomorphic spindle cells and multinucleated giant cells that can stain positively for CD10 and procollagen 1.10
Cutaneous pseudolymphoma is a benign inflammatory response process that stimulates polyclonal T- or B-cell lymphoproliferation. The clinical presentation may appear as localized or disseminated flesh-colored or red papules, infiltrated plaques, and nodules.11 Histopathology will show mixtures of B and T cells along with dendritic cells and macrophages, but irregular vascular structure and dissecting dermal collagen are not involved.
We present an unusual case of CAS with multiple pink nodules on the scalp. Early biopsy of these lesions is important to reach a correct diagnosis and to initiate appropriate treatment.
References
Ishida Y, Otsuka A, Kabashima K. Cutaneous angiosarcoma: update on biology and latest treatment. Curr Opin Oncol. 2018;30:107-112.
Dossett LA, Harrington M, Cruse CW, et al. Cutaneous angiosarcoma. Curr Probl Cancer. 2015;39:258-263.
Albores-Saavedra J, Schwartz AM, Henson DE, et al. Cutaneous angiosarcoma. analysis of 434 cases from the surveillance, epidemiology, and end results program, 1973-2007. Ann Diagn Pathol. 2011;15:93-97.
Guadagnolo BA, Zagars GK, Araujo D, et al. Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp. Head Neck. 2011;33:661-667.
Lindford A, Böhling T, Vaalavirta L, et al. Surgical management of radiation-associated cutaneous breast angiosarcoma. J Plast Reconstr Aesthet Surg. 2011;64:1036-1042.
Costa EPW, Lu.0cena BD, Amin GA, et al. Primary cutaneous follicle center lymphoma. An Bras Dermatol. 2017;92:701-703.
Menon AR, Thomas AS, Suresh N, et al. Cutaneous metastasis: an unusual presenting feature of urologic malignancies. Urol Ann. 2016;8:377-380.
Iorizzo LJ 3rd, Brown MD. Atypical fibroxanthoma: a review of the literature. Dermatol Surg. 2011;37:146-157.
Sarac E, Yuksel M, Turkmen IC, et al. Case for diagnosis. atypical fibroxanthoma. An Bras Dermatol. 2019;94:239-241.
Miguel D, Peckruhn M, Elsner P. Treatment of cutaneous pseudolymphoma: a systematic review. Acta Derm Venereol. 2018;98:310-317.
References
Ishida Y, Otsuka A, Kabashima K. Cutaneous angiosarcoma: update on biology and latest treatment. Curr Opin Oncol. 2018;30:107-112.
Dossett LA, Harrington M, Cruse CW, et al. Cutaneous angiosarcoma. Curr Probl Cancer. 2015;39:258-263.
Albores-Saavedra J, Schwartz AM, Henson DE, et al. Cutaneous angiosarcoma. analysis of 434 cases from the surveillance, epidemiology, and end results program, 1973-2007. Ann Diagn Pathol. 2011;15:93-97.
Guadagnolo BA, Zagars GK, Araujo D, et al. Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp. Head Neck. 2011;33:661-667.
Lindford A, Böhling T, Vaalavirta L, et al. Surgical management of radiation-associated cutaneous breast angiosarcoma. J Plast Reconstr Aesthet Surg. 2011;64:1036-1042.
Costa EPW, Lu.0cena BD, Amin GA, et al. Primary cutaneous follicle center lymphoma. An Bras Dermatol. 2017;92:701-703.
Menon AR, Thomas AS, Suresh N, et al. Cutaneous metastasis: an unusual presenting feature of urologic malignancies. Urol Ann. 2016;8:377-380.
Iorizzo LJ 3rd, Brown MD. Atypical fibroxanthoma: a review of the literature. Dermatol Surg. 2011;37:146-157.
A 67-year-old man presented with pink nodules on the scalp that were enlarging and increasing over the course of 2 months. The patient was otherwise healthy, had no constitutional symptoms such as fever or weight loss, and did not note pruritus or pain. His medications included telmisartan and Salvia miltiorrhiza for hypertension and coronary heart disease, respectively. He had been a heavy smoker for 44 years. Physical examination revealed several dome-shaped, pink nodules with smooth surfaces distributed in an agminated appearance on the scalp. The lesions were indurated and ranged from 1 to 5 cm in diameter.
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We read with great interest the article by Chu et al1 (Cutis. 2021;107:157-159) and commend them for noting the underrepresentation of skin of color (SOC) in the American Academy of Dermatology (AAD) Basic Dermatology Curriculum. The AAD Basic Dermatology Curriculum represents one introductory resource that is ubiquitously utilized by medical students. Herein, we add an analysis of the representation of SOC in the following resources that also comprise the first exposure medical students have to dermatology: Dermatology Clinics Clinical Advisor articles (https://www.clinicaladvisor.com/home/dermatology/dermatology-clinics/), Learn Derm Module (LDM) by VisualDx (https://www.visualdx.com/learnderm/), Lookingbill and Marks’Principles of Dermatology (6th ed)(LB&M),2 and DermNet NZ (https://dermnetnz.org/). We performed a focused search of the DermNet NZ database for images of the following common dermatologic conditions: acne, rosacea, alopecia, urticaria, arthropod bites, blistering diseases (bullous pemphigoid and pemphigus vulgaris), connective tissue diseases (dermatomyositis and lupus), inflammatory conditions (atopic dermatitis, contact dermatitis, and psoriasis), keloids, benign and malignant neoplasms (nevi, seborrheic keratosis, actinic keratosis, basal and squamous cell carcinomas, and melanoma including acral melanoma), bacterial skin infections (impetigo, erysipelas, cellulitis, staphylococcal scalded skin syndrome, and syphilis), fungal infections (dermatophyte infections), and viral skin infections (herpes, molluscum contagiosum, varicella-zoster virus, and warts). We classified images as light (Fitzpatrick phototypes I–IV) or dark (Fitzpatrick phototypes V or VI). We excluded images without visible background skin (eg, images of oral mucosa, genitalia, nails, palms and soles, dermoscopic images, histopathologic images).
We found the representation of SOC in the resources we selected to be as follows: Dermatology Clinics Clinical Advisor articles (70/367 or 19%); LDM (26/150 or 17%); LB&M (52/374 or 14%); DermNet NZ (11/310 or 4%). Representation of SOC in common dermatologic conditions such as actinic keratosis, alopecia, rosacea, urticaria, and warts was entirely absent across all resources. Other common skin diseases were represented in only one of the resources we analyzed: acne (represented only in LB&M, where only 3/11 images of acne were depicted in SOC); contact dermatitis (represented only in LB&M, where only 1/6 images of contact dermatitis were depicted in SOC); psoriasis (represented only on DermNet NZ, where only 2/25 images of psoriasis were depicted in SOC); seborrheic keratosis (represented only in LB&M, where 1/2 images of seborrheic keratosis were depicted in SOC). Furthermore, none of the resources we analyzed depicted malignancy (basal cell carcinoma, squamous cell carcinoma, and melanoma) in SOC. Although the poor representation of SOC in malignancies can be explained by the predilection of skin cancer for light skin, other dermatologic conditions that are more common in SOC also were poorly represented in these resources in SOC: acral melanoma, not represented in any of the resources we analyzed; subacute cutaneous lupus erythematosus and systemic lupus erythematosus, also not represented in any of the resources we analyzed; keloids, represented only in LB&M.
Although no study has investigated the true prevalence of Fitzpatrick phototypes in the United States, He et al3 demonstrated the prevalence of Fitzpatrick phototypes V and VI to be 25.0% and 18.8%, respectively, in an ethnically diverse study of 3386 participants. Indeed, the representation of SOC in the resources we analyzed falls short of this plausible estimate of SOC in an increasingly diverse US population.
Our work adds to the growing body of literature exposing the deficiencies in SOC representation in dermatology. As Lester et al4 noted, such poor representation of SOC is deleterious not just to patients, who may be misdiagnosed, but also more generally to the integrity of the field of dermatology. Moreover, our study, which analyzes introductory resources referenced by the junior medical student, highlights a potential danger of poor SOC representation for trainees—limited exposure to SOC may leave medical students unprepared to recognize lesions in SOC during clerkships and residency. Furthermore, we note an additional concern with minimal SOC representation in online modules such as the AAD and LDM module as well as online databases such as DermNet NZ; images from these resources may be used as training sets for machine learning (ML) software (indeed, DermNet NZ has been used as a training set for ML programs5). However, if data sets with poor representation of SOC are used to train ML algorithms, then ML software may be unable to recognize lesions in SOC.6 Thus, inadequate representation of SOC in online modules and databases may exacerbate existing inequities in dermatology.
To address the paucity of SOC representation, students can be directed to resources devoted to depicting SOC; however, as discussed eloquently by Chu et al,1 an attempt to update existing resources also must be made. The senior author in our study (S.J.K.) embraced such an approach, updating the dermatology lectures given to medical students to include more images of SOC. Such a top-down approach may represent a major step in dismantling the systemic biases that pervade dermatology.
A limitation of our analysis was use of the Fitzpatrick scale, which was conceived as a phenotypic scale to assess cutaneous responses to UV irradiation.7 Although it is the most commonly used scale to describe race/ethnicity and/or constitute skin color, it is not possible to include all non-White skin types and classify strictly under this umbrella term.
References
1. Chu B, Fathy R, Onyekaba G, et al. Distribution of skin-type diversity in photographs in AAD online educational modules. Cutis. 2021;107:157-159. doi:10.12788/cutis.0196
2. Marks JG Jr, Miller JJ. Lookingbill and Marks’ Principles of Dermatology. 6th ed. Saunders Elsevier; 2018.
3. He SY, McCulloch CE, Boscardin WJ, et al. Self-reported pigmentary phenotypes and race are significant but incomplete predictors of Fitzpatrick skin phototype in an ethnically diverse population. J Am Acad Dermatol. 2014;71:731-737. doi:10.1016/j.jaad.2014.05.023
4. Lester JC, Taylor SC, Chren M-M. Under‐representation of skin of colour in dermatology images: not just an educational issue. Br J Dermatol. 2019;180:1521-1522. doi:10.1111/bjd.17608
5. Aggarwal P. Data augmentation in dermatology image recognition using machine learning. Skin Res Technol. 2019;25:815-820. doi:10.1111/srt.12726
6. Adamson AS, Smith A. Machine learning and health care disparities in dermatology. JAMA Dermatol. 2018;154:1247-1248. doi:10.1001/jamadermatol.2018.2348
7. Ware OR, Dawson JE, Shinohara MM, et al. Racial limitations of Fitzpatrick skin type. Cutis. 2020;105:77-80.
Authors’ Response
We thank Mr. Joshi and Dr. Kim for their reply to our article and their added contribution to the literature on inadequate representation of skin of color (SOC) in dermatology educational materials. In recent years, multiple analyses have reviewed textbooks and popular online resources for SOC representation.1 These resources encompass all levels of education—from the laypatient to the medical student, and to residency and beyond—demonstrating the significant challenges to overcome.
In addition, as Mr. Joshi and Dr. Kim state, the potential for these inadequately representative resources to serve as training data for prediction and classification tools adds further urgency to the broader task at hand, as we do not wish to perpetuate disparities. Several tools already exist, including Derm Assist, a recent Google-produced tool that suggests a list of diagnoses from patient-provided images.2 Although Derm Assist has been marked as a CE Class I (low risk) medical device in the European Union, the original research it is built on relied on training data with low representation of darker skin types (2.7% Fitzpatrick V and 0% Fitzpatrick VI),3 drawing concern for its generalizability.
These concerns about SOC representation are not new; dermatology advocates, scholars, and organizations such as the Skin of Color Society have been working to address these deficiencies for many years, contributing to education (including writing of resources and textbooks) and academic research. This work continues today. For instance, Lester et al4 described best practices for clinical photography in SOC; this guidance was not yet published at the time of our original submission. Not only should dermatology strive for increased quantity of representation but also quality. This metric is particularly important if the images are intended not just for education but also for use as training data for prediction and classification tools.
Examples of more recent actions at the organizational level include the American Academy of Dermatology (AAD) announcing a 3-year plan to promote diversity, equity, and inclusion5 and VisualDx establishing #ProjectIMPACT, a collaboration to reduce health care biases in SOC.6 In the AAD 3-year plan, one goal is to “[i]ncrease use of images reflecting full spectrum of skin types and highlight topics on skin of color, health disparities, and cultural competency across all AAD education.”5 Although not specifically mentioned, we hope that the AAD has included updating the Basic Dermatology Curriculum, given its inadequate SOC representation, as part of its short-term goals. The greater recognition of these issues through more prevalent analyses published in leading dermatology journals is encouraging, and we hope both that improvements can be successfully implemented and that future studies will reveal improvements in representation.
Brian Chu, BS; Ramie Fathy, AB; Ginikanwa Onyekaba, BS; Jules B. Lipoff, MD
From the Perelman School of Medicine, University of Pennsylvania, Philadelphia. Dr. Lipoff is from the Department of Dermatology and the Leonard Davis Institute of Health Economics.
The authors report no conflict of interest.
Correspondence: Jules B. Lipoff, MD, Department of Dermatology, University of Pennsylvania, Penn Medicine University City, 3737 Market St, Ste 1100, Philadelphia, PA 19104 (jules.lipoff@pennmedicine.upenn.edu).
References
1. Perlman KL, Williams NM, Egbeto IA, et al. Skin of color lacks representation in medical student resources: a cross-sectional study. Int J Womens Dermatol. 2021;7:195-196. doi:10.1016/j.ijwd.2020.12.018
2. Bui P, Liu Y. Using AI to help find answers to common skin conditions. Published May 18, 2021. Accessed June 12, 2021. https://blog.google/technology/health/ai-dermatology-preview-io-2021
3. Liu Y, Jain A, Eng C, et al. A deep learning system for differential diagnosis of skin diseases. Nature Medicine. 2020;26:900-908. doi:10.1038/s41591-020-0842-3
4. Lester JC, Clark L, Linos E, et al. Clinical photography in skin of colour: tips and best practices. Br J Dermatol. 2021;184:1177-1179. doi:10.1111/bjd.19811
5. American Academy of Dermatology Association. Diversity in dermatology: diversity committee approved plan 2021-2023. Published January 26, 2021. Accessed June 24, 2021. https://assets.ctfassets.net/1ny4yoiyrqia/xQgnCE6ji5skUlcZQHS2b/65f0a9072811e11afcc33d043e02cd4d/DEI_Plan.pdf
6. VisualDx. #ProjectIMPACT. Accessed June 24, 2021. https://www.visualdx.com/projectimpact/
We read with great interest the article by Chu et al1 (Cutis. 2021;107:157-159) and commend them for noting the underrepresentation of skin of color (SOC) in the American Academy of Dermatology (AAD) Basic Dermatology Curriculum. The AAD Basic Dermatology Curriculum represents one introductory resource that is ubiquitously utilized by medical students. Herein, we add an analysis of the representation of SOC in the following resources that also comprise the first exposure medical students have to dermatology: Dermatology Clinics Clinical Advisor articles (https://www.clinicaladvisor.com/home/dermatology/dermatology-clinics/), Learn Derm Module (LDM) by VisualDx (https://www.visualdx.com/learnderm/), Lookingbill and Marks’Principles of Dermatology (6th ed)(LB&M),2 and DermNet NZ (https://dermnetnz.org/). We performed a focused search of the DermNet NZ database for images of the following common dermatologic conditions: acne, rosacea, alopecia, urticaria, arthropod bites, blistering diseases (bullous pemphigoid and pemphigus vulgaris), connective tissue diseases (dermatomyositis and lupus), inflammatory conditions (atopic dermatitis, contact dermatitis, and psoriasis), keloids, benign and malignant neoplasms (nevi, seborrheic keratosis, actinic keratosis, basal and squamous cell carcinomas, and melanoma including acral melanoma), bacterial skin infections (impetigo, erysipelas, cellulitis, staphylococcal scalded skin syndrome, and syphilis), fungal infections (dermatophyte infections), and viral skin infections (herpes, molluscum contagiosum, varicella-zoster virus, and warts). We classified images as light (Fitzpatrick phototypes I–IV) or dark (Fitzpatrick phototypes V or VI). We excluded images without visible background skin (eg, images of oral mucosa, genitalia, nails, palms and soles, dermoscopic images, histopathologic images).
We found the representation of SOC in the resources we selected to be as follows: Dermatology Clinics Clinical Advisor articles (70/367 or 19%); LDM (26/150 or 17%); LB&M (52/374 or 14%); DermNet NZ (11/310 or 4%). Representation of SOC in common dermatologic conditions such as actinic keratosis, alopecia, rosacea, urticaria, and warts was entirely absent across all resources. Other common skin diseases were represented in only one of the resources we analyzed: acne (represented only in LB&M, where only 3/11 images of acne were depicted in SOC); contact dermatitis (represented only in LB&M, where only 1/6 images of contact dermatitis were depicted in SOC); psoriasis (represented only on DermNet NZ, where only 2/25 images of psoriasis were depicted in SOC); seborrheic keratosis (represented only in LB&M, where 1/2 images of seborrheic keratosis were depicted in SOC). Furthermore, none of the resources we analyzed depicted malignancy (basal cell carcinoma, squamous cell carcinoma, and melanoma) in SOC. Although the poor representation of SOC in malignancies can be explained by the predilection of skin cancer for light skin, other dermatologic conditions that are more common in SOC also were poorly represented in these resources in SOC: acral melanoma, not represented in any of the resources we analyzed; subacute cutaneous lupus erythematosus and systemic lupus erythematosus, also not represented in any of the resources we analyzed; keloids, represented only in LB&M.
Although no study has investigated the true prevalence of Fitzpatrick phototypes in the United States, He et al3 demonstrated the prevalence of Fitzpatrick phototypes V and VI to be 25.0% and 18.8%, respectively, in an ethnically diverse study of 3386 participants. Indeed, the representation of SOC in the resources we analyzed falls short of this plausible estimate of SOC in an increasingly diverse US population.
Our work adds to the growing body of literature exposing the deficiencies in SOC representation in dermatology. As Lester et al4 noted, such poor representation of SOC is deleterious not just to patients, who may be misdiagnosed, but also more generally to the integrity of the field of dermatology. Moreover, our study, which analyzes introductory resources referenced by the junior medical student, highlights a potential danger of poor SOC representation for trainees—limited exposure to SOC may leave medical students unprepared to recognize lesions in SOC during clerkships and residency. Furthermore, we note an additional concern with minimal SOC representation in online modules such as the AAD and LDM module as well as online databases such as DermNet NZ; images from these resources may be used as training sets for machine learning (ML) software (indeed, DermNet NZ has been used as a training set for ML programs5). However, if data sets with poor representation of SOC are used to train ML algorithms, then ML software may be unable to recognize lesions in SOC.6 Thus, inadequate representation of SOC in online modules and databases may exacerbate existing inequities in dermatology.
To address the paucity of SOC representation, students can be directed to resources devoted to depicting SOC; however, as discussed eloquently by Chu et al,1 an attempt to update existing resources also must be made. The senior author in our study (S.J.K.) embraced such an approach, updating the dermatology lectures given to medical students to include more images of SOC. Such a top-down approach may represent a major step in dismantling the systemic biases that pervade dermatology.
A limitation of our analysis was use of the Fitzpatrick scale, which was conceived as a phenotypic scale to assess cutaneous responses to UV irradiation.7 Although it is the most commonly used scale to describe race/ethnicity and/or constitute skin color, it is not possible to include all non-White skin types and classify strictly under this umbrella term.
References
1. Chu B, Fathy R, Onyekaba G, et al. Distribution of skin-type diversity in photographs in AAD online educational modules. Cutis. 2021;107:157-159. doi:10.12788/cutis.0196
2. Marks JG Jr, Miller JJ. Lookingbill and Marks’ Principles of Dermatology. 6th ed. Saunders Elsevier; 2018.
3. He SY, McCulloch CE, Boscardin WJ, et al. Self-reported pigmentary phenotypes and race are significant but incomplete predictors of Fitzpatrick skin phototype in an ethnically diverse population. J Am Acad Dermatol. 2014;71:731-737. doi:10.1016/j.jaad.2014.05.023
4. Lester JC, Taylor SC, Chren M-M. Under‐representation of skin of colour in dermatology images: not just an educational issue. Br J Dermatol. 2019;180:1521-1522. doi:10.1111/bjd.17608
5. Aggarwal P. Data augmentation in dermatology image recognition using machine learning. Skin Res Technol. 2019;25:815-820. doi:10.1111/srt.12726
6. Adamson AS, Smith A. Machine learning and health care disparities in dermatology. JAMA Dermatol. 2018;154:1247-1248. doi:10.1001/jamadermatol.2018.2348
7. Ware OR, Dawson JE, Shinohara MM, et al. Racial limitations of Fitzpatrick skin type. Cutis. 2020;105:77-80.
Authors’ Response
We thank Mr. Joshi and Dr. Kim for their reply to our article and their added contribution to the literature on inadequate representation of skin of color (SOC) in dermatology educational materials. In recent years, multiple analyses have reviewed textbooks and popular online resources for SOC representation.1 These resources encompass all levels of education—from the laypatient to the medical student, and to residency and beyond—demonstrating the significant challenges to overcome.
In addition, as Mr. Joshi and Dr. Kim state, the potential for these inadequately representative resources to serve as training data for prediction and classification tools adds further urgency to the broader task at hand, as we do not wish to perpetuate disparities. Several tools already exist, including Derm Assist, a recent Google-produced tool that suggests a list of diagnoses from patient-provided images.2 Although Derm Assist has been marked as a CE Class I (low risk) medical device in the European Union, the original research it is built on relied on training data with low representation of darker skin types (2.7% Fitzpatrick V and 0% Fitzpatrick VI),3 drawing concern for its generalizability.
These concerns about SOC representation are not new; dermatology advocates, scholars, and organizations such as the Skin of Color Society have been working to address these deficiencies for many years, contributing to education (including writing of resources and textbooks) and academic research. This work continues today. For instance, Lester et al4 described best practices for clinical photography in SOC; this guidance was not yet published at the time of our original submission. Not only should dermatology strive for increased quantity of representation but also quality. This metric is particularly important if the images are intended not just for education but also for use as training data for prediction and classification tools.
Examples of more recent actions at the organizational level include the American Academy of Dermatology (AAD) announcing a 3-year plan to promote diversity, equity, and inclusion5 and VisualDx establishing #ProjectIMPACT, a collaboration to reduce health care biases in SOC.6 In the AAD 3-year plan, one goal is to “[i]ncrease use of images reflecting full spectrum of skin types and highlight topics on skin of color, health disparities, and cultural competency across all AAD education.”5 Although not specifically mentioned, we hope that the AAD has included updating the Basic Dermatology Curriculum, given its inadequate SOC representation, as part of its short-term goals. The greater recognition of these issues through more prevalent analyses published in leading dermatology journals is encouraging, and we hope both that improvements can be successfully implemented and that future studies will reveal improvements in representation.
Brian Chu, BS; Ramie Fathy, AB; Ginikanwa Onyekaba, BS; Jules B. Lipoff, MD
From the Perelman School of Medicine, University of Pennsylvania, Philadelphia. Dr. Lipoff is from the Department of Dermatology and the Leonard Davis Institute of Health Economics.
The authors report no conflict of interest.
Correspondence: Jules B. Lipoff, MD, Department of Dermatology, University of Pennsylvania, Penn Medicine University City, 3737 Market St, Ste 1100, Philadelphia, PA 19104 (jules.lipoff@pennmedicine.upenn.edu).
References
1. Perlman KL, Williams NM, Egbeto IA, et al. Skin of color lacks representation in medical student resources: a cross-sectional study. Int J Womens Dermatol. 2021;7:195-196. doi:10.1016/j.ijwd.2020.12.018
2. Bui P, Liu Y. Using AI to help find answers to common skin conditions. Published May 18, 2021. Accessed June 12, 2021. https://blog.google/technology/health/ai-dermatology-preview-io-2021
3. Liu Y, Jain A, Eng C, et al. A deep learning system for differential diagnosis of skin diseases. Nature Medicine. 2020;26:900-908. doi:10.1038/s41591-020-0842-3
4. Lester JC, Clark L, Linos E, et al. Clinical photography in skin of colour: tips and best practices. Br J Dermatol. 2021;184:1177-1179. doi:10.1111/bjd.19811
5. American Academy of Dermatology Association. Diversity in dermatology: diversity committee approved plan 2021-2023. Published January 26, 2021. Accessed June 24, 2021. https://assets.ctfassets.net/1ny4yoiyrqia/xQgnCE6ji5skUlcZQHS2b/65f0a9072811e11afcc33d043e02cd4d/DEI_Plan.pdf
6. VisualDx. #ProjectIMPACT. Accessed June 24, 2021. https://www.visualdx.com/projectimpact/
To the Editor:
We read with great interest the article by Chu et al1 (Cutis. 2021;107:157-159) and commend them for noting the underrepresentation of skin of color (SOC) in the American Academy of Dermatology (AAD) Basic Dermatology Curriculum. The AAD Basic Dermatology Curriculum represents one introductory resource that is ubiquitously utilized by medical students. Herein, we add an analysis of the representation of SOC in the following resources that also comprise the first exposure medical students have to dermatology: Dermatology Clinics Clinical Advisor articles (https://www.clinicaladvisor.com/home/dermatology/dermatology-clinics/), Learn Derm Module (LDM) by VisualDx (https://www.visualdx.com/learnderm/), Lookingbill and Marks’Principles of Dermatology (6th ed)(LB&M),2 and DermNet NZ (https://dermnetnz.org/). We performed a focused search of the DermNet NZ database for images of the following common dermatologic conditions: acne, rosacea, alopecia, urticaria, arthropod bites, blistering diseases (bullous pemphigoid and pemphigus vulgaris), connective tissue diseases (dermatomyositis and lupus), inflammatory conditions (atopic dermatitis, contact dermatitis, and psoriasis), keloids, benign and malignant neoplasms (nevi, seborrheic keratosis, actinic keratosis, basal and squamous cell carcinomas, and melanoma including acral melanoma), bacterial skin infections (impetigo, erysipelas, cellulitis, staphylococcal scalded skin syndrome, and syphilis), fungal infections (dermatophyte infections), and viral skin infections (herpes, molluscum contagiosum, varicella-zoster virus, and warts). We classified images as light (Fitzpatrick phototypes I–IV) or dark (Fitzpatrick phototypes V or VI). We excluded images without visible background skin (eg, images of oral mucosa, genitalia, nails, palms and soles, dermoscopic images, histopathologic images).
We found the representation of SOC in the resources we selected to be as follows: Dermatology Clinics Clinical Advisor articles (70/367 or 19%); LDM (26/150 or 17%); LB&M (52/374 or 14%); DermNet NZ (11/310 or 4%). Representation of SOC in common dermatologic conditions such as actinic keratosis, alopecia, rosacea, urticaria, and warts was entirely absent across all resources. Other common skin diseases were represented in only one of the resources we analyzed: acne (represented only in LB&M, where only 3/11 images of acne were depicted in SOC); contact dermatitis (represented only in LB&M, where only 1/6 images of contact dermatitis were depicted in SOC); psoriasis (represented only on DermNet NZ, where only 2/25 images of psoriasis were depicted in SOC); seborrheic keratosis (represented only in LB&M, where 1/2 images of seborrheic keratosis were depicted in SOC). Furthermore, none of the resources we analyzed depicted malignancy (basal cell carcinoma, squamous cell carcinoma, and melanoma) in SOC. Although the poor representation of SOC in malignancies can be explained by the predilection of skin cancer for light skin, other dermatologic conditions that are more common in SOC also were poorly represented in these resources in SOC: acral melanoma, not represented in any of the resources we analyzed; subacute cutaneous lupus erythematosus and systemic lupus erythematosus, also not represented in any of the resources we analyzed; keloids, represented only in LB&M.
Although no study has investigated the true prevalence of Fitzpatrick phototypes in the United States, He et al3 demonstrated the prevalence of Fitzpatrick phototypes V and VI to be 25.0% and 18.8%, respectively, in an ethnically diverse study of 3386 participants. Indeed, the representation of SOC in the resources we analyzed falls short of this plausible estimate of SOC in an increasingly diverse US population.
Our work adds to the growing body of literature exposing the deficiencies in SOC representation in dermatology. As Lester et al4 noted, such poor representation of SOC is deleterious not just to patients, who may be misdiagnosed, but also more generally to the integrity of the field of dermatology. Moreover, our study, which analyzes introductory resources referenced by the junior medical student, highlights a potential danger of poor SOC representation for trainees—limited exposure to SOC may leave medical students unprepared to recognize lesions in SOC during clerkships and residency. Furthermore, we note an additional concern with minimal SOC representation in online modules such as the AAD and LDM module as well as online databases such as DermNet NZ; images from these resources may be used as training sets for machine learning (ML) software (indeed, DermNet NZ has been used as a training set for ML programs5). However, if data sets with poor representation of SOC are used to train ML algorithms, then ML software may be unable to recognize lesions in SOC.6 Thus, inadequate representation of SOC in online modules and databases may exacerbate existing inequities in dermatology.
To address the paucity of SOC representation, students can be directed to resources devoted to depicting SOC; however, as discussed eloquently by Chu et al,1 an attempt to update existing resources also must be made. The senior author in our study (S.J.K.) embraced such an approach, updating the dermatology lectures given to medical students to include more images of SOC. Such a top-down approach may represent a major step in dismantling the systemic biases that pervade dermatology.
A limitation of our analysis was use of the Fitzpatrick scale, which was conceived as a phenotypic scale to assess cutaneous responses to UV irradiation.7 Although it is the most commonly used scale to describe race/ethnicity and/or constitute skin color, it is not possible to include all non-White skin types and classify strictly under this umbrella term.
References
1. Chu B, Fathy R, Onyekaba G, et al. Distribution of skin-type diversity in photographs in AAD online educational modules. Cutis. 2021;107:157-159. doi:10.12788/cutis.0196
2. Marks JG Jr, Miller JJ. Lookingbill and Marks’ Principles of Dermatology. 6th ed. Saunders Elsevier; 2018.
3. He SY, McCulloch CE, Boscardin WJ, et al. Self-reported pigmentary phenotypes and race are significant but incomplete predictors of Fitzpatrick skin phototype in an ethnically diverse population. J Am Acad Dermatol. 2014;71:731-737. doi:10.1016/j.jaad.2014.05.023
4. Lester JC, Taylor SC, Chren M-M. Under‐representation of skin of colour in dermatology images: not just an educational issue. Br J Dermatol. 2019;180:1521-1522. doi:10.1111/bjd.17608
5. Aggarwal P. Data augmentation in dermatology image recognition using machine learning. Skin Res Technol. 2019;25:815-820. doi:10.1111/srt.12726
6. Adamson AS, Smith A. Machine learning and health care disparities in dermatology. JAMA Dermatol. 2018;154:1247-1248. doi:10.1001/jamadermatol.2018.2348
7. Ware OR, Dawson JE, Shinohara MM, et al. Racial limitations of Fitzpatrick skin type. Cutis. 2020;105:77-80.
Authors’ Response
We thank Mr. Joshi and Dr. Kim for their reply to our article and their added contribution to the literature on inadequate representation of skin of color (SOC) in dermatology educational materials. In recent years, multiple analyses have reviewed textbooks and popular online resources for SOC representation.1 These resources encompass all levels of education—from the laypatient to the medical student, and to residency and beyond—demonstrating the significant challenges to overcome.
In addition, as Mr. Joshi and Dr. Kim state, the potential for these inadequately representative resources to serve as training data for prediction and classification tools adds further urgency to the broader task at hand, as we do not wish to perpetuate disparities. Several tools already exist, including Derm Assist, a recent Google-produced tool that suggests a list of diagnoses from patient-provided images.2 Although Derm Assist has been marked as a CE Class I (low risk) medical device in the European Union, the original research it is built on relied on training data with low representation of darker skin types (2.7% Fitzpatrick V and 0% Fitzpatrick VI),3 drawing concern for its generalizability.
These concerns about SOC representation are not new; dermatology advocates, scholars, and organizations such as the Skin of Color Society have been working to address these deficiencies for many years, contributing to education (including writing of resources and textbooks) and academic research. This work continues today. For instance, Lester et al4 described best practices for clinical photography in SOC; this guidance was not yet published at the time of our original submission. Not only should dermatology strive for increased quantity of representation but also quality. This metric is particularly important if the images are intended not just for education but also for use as training data for prediction and classification tools.
Examples of more recent actions at the organizational level include the American Academy of Dermatology (AAD) announcing a 3-year plan to promote diversity, equity, and inclusion5 and VisualDx establishing #ProjectIMPACT, a collaboration to reduce health care biases in SOC.6 In the AAD 3-year plan, one goal is to “[i]ncrease use of images reflecting full spectrum of skin types and highlight topics on skin of color, health disparities, and cultural competency across all AAD education.”5 Although not specifically mentioned, we hope that the AAD has included updating the Basic Dermatology Curriculum, given its inadequate SOC representation, as part of its short-term goals. The greater recognition of these issues through more prevalent analyses published in leading dermatology journals is encouraging, and we hope both that improvements can be successfully implemented and that future studies will reveal improvements in representation.
Brian Chu, BS; Ramie Fathy, AB; Ginikanwa Onyekaba, BS; Jules B. Lipoff, MD
From the Perelman School of Medicine, University of Pennsylvania, Philadelphia. Dr. Lipoff is from the Department of Dermatology and the Leonard Davis Institute of Health Economics.
The authors report no conflict of interest.
Correspondence: Jules B. Lipoff, MD, Department of Dermatology, University of Pennsylvania, Penn Medicine University City, 3737 Market St, Ste 1100, Philadelphia, PA 19104 (jules.lipoff@pennmedicine.upenn.edu).
References
1. Perlman KL, Williams NM, Egbeto IA, et al. Skin of color lacks representation in medical student resources: a cross-sectional study. Int J Womens Dermatol. 2021;7:195-196. doi:10.1016/j.ijwd.2020.12.018
2. Bui P, Liu Y. Using AI to help find answers to common skin conditions. Published May 18, 2021. Accessed June 12, 2021. https://blog.google/technology/health/ai-dermatology-preview-io-2021
3. Liu Y, Jain A, Eng C, et al. A deep learning system for differential diagnosis of skin diseases. Nature Medicine. 2020;26:900-908. doi:10.1038/s41591-020-0842-3
4. Lester JC, Clark L, Linos E, et al. Clinical photography in skin of colour: tips and best practices. Br J Dermatol. 2021;184:1177-1179. doi:10.1111/bjd.19811
5. American Academy of Dermatology Association. Diversity in dermatology: diversity committee approved plan 2021-2023. Published January 26, 2021. Accessed June 24, 2021. https://assets.ctfassets.net/1ny4yoiyrqia/xQgnCE6ji5skUlcZQHS2b/65f0a9072811e11afcc33d043e02cd4d/DEI_Plan.pdf
6. VisualDx. #ProjectIMPACT. Accessed June 24, 2021. https://www.visualdx.com/projectimpact/
Rosacea fulminans (RF) is a rare facial dermatosis characterized by its fulminating course. 1 It presents with superficial and deep-seated papules, pustules, and nodules combined with an intense reddish or cyanotic erythema localized to the face. Furthermore, there is an absence of comedones and involvement of the chest or back. 2 Rosacea fulminans primarily affects women and often is, but not always, proceeded by seborrhea, chronic acne vulgaris, or rosacea. Although the etiology of RF remains unknown, immunologic, hormonal, and vascular factors have been implicated. 3 We report a case of RF in a pregnant patient with a history of mild acne as a teenager that was long ago resolved.
Case Report
A 32-year-old pregnant woman (10 weeks’ gestation) presented with a rapidly progressing inflammatory disorder of the face of 1 month’s duration. The lesions developed 3 weeks after beginning progesterone therapy (200 mg vaginal suppository) for infertility due to polycystic ovary syndrome. Despite discontinuing progesterone for the last month, the patient’s lesions had dramatically worsened (Figure 1). Empiric cephalosporin treatment prescribed by her primary care physician yielded no improvement. Physical examination at the current presentation revealed erythematous nodules and pustules all over the face, coalescing into large thick plaques on the patient’s right cheek and chin. Submental nodes were palpable and tender. Based on the initial clinical findings, acne conglobata secondary to progesterone therapy was considered. The patient was given intralesional triamcinolone (2.5 mg/cc) injections to all larger nodules and several blue light treatments.
Figure 1. Rosacea fulminans in a pregnant woman at presentation (10 weeks’ gestation).
The injected areas had improved 5 days after the initial visit; however, the chin and right paranasal cheek developed even more nodules and papules coalescing into large plaques. After consulting the patient’s obstetrician, prednisone (20 mg once daily) was initiated. Three weeks later, the patient’s nodular lesions had improved, but there was a showering of more than 100 pustules and increased general erythema of the entire face (Figure 2). Crotamiton cream 10% (every day before noon), ivermectin cream 1% (every night at bedtime), and sodium sulfacetamide cleanser 10% once daily were added to the treatment plan.
Figure 2. Three weeks after starting prednisone, there was a showering of more than 100 pustules and increased general erythema of the entire face due to rosacea fulminans.
At 16 weeks’ gestation, there was slight improvement; however, there was still erythema on the entire face with scattered pustules and multiple papules and nodules. Many small ice-pick scars were seen on the cheeks and forehead. No comedones were observed. A punch biopsy of an intact papule showed a prominent inflammatory infiltrate with granulomatous reaction and numerous neutrophils predominantly affecting hair follicles. Based on the clinical presentation and histopathology, a diagnosis of RF was made. Azithromycin (250 mg once daily) and metronidazole cream 0.75% twice daily were added. Two weeks later there were fewer nodules but many papules, edema, and intense erythema. The prednisone dosage was increased to 40 mg once daily. Two weeks later, the patient showed improvement with fewer lesions, less edema, and less erythema. The patient was instructed to finish the azithromycin course and discontinue use. At 28 weeks’ gestation, a prednisone taper was started with the intention to reduce the daily dose by delivery.
The patient delivered a healthy girl (birth weight, 1.985 kg) prematurely at 34 weeks’ gestation. At 2 months postpartum, the patient’s existing lesions continued to spontaneously improve; however, she still had numerous nodules and papules and continued to develop new lesions and form additional scars. Isotretinoin was instituted at 3 months postpartum upon cessation of nursing. Three months later (40 mg/d isotretinoin), the patient was nearly clear. At 8 months postpartum, isotretinoin was discontinued after a course of 150 mg/kg.
Comment
Rosacea fulminans initially was called pyoderma faciale but was later regarded as a severe form of rosacea and was renamed rosacea fulminans.2 According to a PubMed search of articles indexed for MEDLINE using the terms pregnancy and rosacea fulminans or pyoderma faciale, we identified 12 publications reporting 20 cases of RF associated with pregnancy (Table). Although there is no substantial evidence regarding the exact mechanism, these cases indicate that pregnancy can be an exacerbating or causative factor in the pathogenesis of RF.
In addition to pregnancy, RF has been associated with inflammatory bowel disease, thyroid and liver disease, erythema nodosum, and severe emotional trauma. However, no organism has been consistently isolated, and no evidence of family history has been reported.1 Histopathologic findings are dependent on the stage of disease. Massive infiltrates of neutrophils may be observed in early stages. In older lesions, infiltrates take the form of epithelioid cell granulomas.2
Treatment of RF during pregnancy is challenging. Early and aggressive treatment with retinoids, tetracycline antibiotics, antiandrogenic contraceptives, and dapsone is recommended in patients who are not pregnant; these therapies are all contraindicated in pregnancy. Topical steroids can be safely used; however, systemic steroids usually are required to control RF. The use of systemic steroids can only be justified if the risks for intrauterine growth retardation, maternal diabetes mellitus, and hypertension outweigh the benefits of treating this severe disfiguring skin condition.10 A study by Bakar et al13 indicated that azithromycin is an effective and safe alternative in the treatment of RF. It has a superior pharmacokinetic profile compared to other macrolides and does not pose increased risks for congenital malformation or miscarriage. Because of the concomitant use of both azithromycin and prednisone, it is not possible to determine which had the larger role in the patient’s improvement.
Isotretinoin therapy in our patient led to substantial improvement of RF. Time will tell if the response will be durable. Also unknown is the risk for recurrence with subsequent pregnancies, which has not been reported in the literature. Although it is difficult to confidently say that pregnancy was the inciting factor in this patient’s RF, this case certainly provides more evidence for a link between pregnancy and RF.
References
Jarrett R, Gonsalves R, Anstey AV. Differing obstetric outcomes of rosacea fulminans in pregnancy: report of three cases with review of pathogenesis and management. Clin Exp Dermatol. 2010;35:888-891. doi:10.1111/j.1365-2230.2010.03846.x
Ferahbas A, Utas S, Mistik S, et al. Rosacea fulminans in pregnancy: case report and review of the literature. Am J Clin Dermatol. 2006;7:141-144. doi:10.2165/00128071-200607020-00007
Fuentelsaz V, Ara M, Corredera C, et al. Rosacea fulminans in pregnancy: successful treatment with azithromycin. Clin Exp Dermatol. 2011;36:674-676. doi:10.1111/j.1365-2230.2011.04042.x
Garayar Cantero M, Garabito Solovera E, Aguado García Á, et al. Use of permethrin in the treatment of rosacea fulminans during pregnancy: one case report. Dermatol Ther. 2020;33:E13436. doi:10.1111/dth.13436
Demir O, Tas IS, Gunay B, et al. A rare dermatologic disease in pregnancy: rosacea fulminans—case report and review of the literature. Open Access Maced J Med Sci. 2018;6:1438-1441. doi:10.3889/oamjms.2018.267
Markou AG, Alessandrini V, Muray JM, et al. Rosacea fulminans during pregnancy. Clin Exp Obstet Gynecol. 2017;44:157-159.
Haenen CCP, Kouwenhoven STP, van Doorn R. Rosacea fulminans in pregnancy [in Dutch]. Ned Tijdschr Geneeskd. 2015;159:A8334.
de Morais e Silva FA, Bonassi M, Steiner D, et al. Rosacea fulminans in pregnancy with ocular perforation. J Dtsch Dermatol Ges. 2011;9:542-543. doi:10.1111/j.1610-0387.2011.07616.x
Cisse M, Maruani A, Bré C. Rosacea fulminans in the early course of a pregnancy by in vitro fertilization with embryo transfer [in French]. Ann Dermatol Venereol. 2008;135:675-678. doi:10.1016/j.annder.2008.04.015
Lewis VJ, Holme SA, Wright A, et al. Rosacea fulminans in pregnancy. Br J Dermatol. 2004;151:917-919. doi:10.1111/j.1365-2133.2004.06190.x
Plewig G, Jansen T, Kligman AM. Pyoderma faciale. a review and report of 20 additional cases: is it rosacea? Arch Dermatol. 1992;128:1611-1617. doi:10.1001/archderm.128.12.1611
Massa MC, Su WP. Pyoderma faciale: a clinical study of twenty-nine patients. J Am Acad Dermatol. 1982;6:84-91. doi:10.1016/s0190-9622(82)70008-8
Bakar O, Demirçay Z, Gürbüz O. Therapeutic potential of azithromycin in rosacea. Int J Dermatol. 2004;43:151-154. doi:10.1111/j.1365-4632.2004.01958.x
Mr. Ranpariya is from Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey. Dr. Baldwin is from Acne Treatment and Research Center, Morristown, New Jersey.
Mr. Ranpariya is from Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey. Dr. Baldwin is from Acne Treatment and Research Center, Morristown, New Jersey.
Mr. Ranpariya is from Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey. Dr. Baldwin is from Acne Treatment and Research Center, Morristown, New Jersey.
Rosacea fulminans (RF) is a rare facial dermatosis characterized by its fulminating course. 1 It presents with superficial and deep-seated papules, pustules, and nodules combined with an intense reddish or cyanotic erythema localized to the face. Furthermore, there is an absence of comedones and involvement of the chest or back. 2 Rosacea fulminans primarily affects women and often is, but not always, proceeded by seborrhea, chronic acne vulgaris, or rosacea. Although the etiology of RF remains unknown, immunologic, hormonal, and vascular factors have been implicated. 3 We report a case of RF in a pregnant patient with a history of mild acne as a teenager that was long ago resolved.
Case Report
A 32-year-old pregnant woman (10 weeks’ gestation) presented with a rapidly progressing inflammatory disorder of the face of 1 month’s duration. The lesions developed 3 weeks after beginning progesterone therapy (200 mg vaginal suppository) for infertility due to polycystic ovary syndrome. Despite discontinuing progesterone for the last month, the patient’s lesions had dramatically worsened (Figure 1). Empiric cephalosporin treatment prescribed by her primary care physician yielded no improvement. Physical examination at the current presentation revealed erythematous nodules and pustules all over the face, coalescing into large thick plaques on the patient’s right cheek and chin. Submental nodes were palpable and tender. Based on the initial clinical findings, acne conglobata secondary to progesterone therapy was considered. The patient was given intralesional triamcinolone (2.5 mg/cc) injections to all larger nodules and several blue light treatments.
Figure 1. Rosacea fulminans in a pregnant woman at presentation (10 weeks’ gestation).
The injected areas had improved 5 days after the initial visit; however, the chin and right paranasal cheek developed even more nodules and papules coalescing into large plaques. After consulting the patient’s obstetrician, prednisone (20 mg once daily) was initiated. Three weeks later, the patient’s nodular lesions had improved, but there was a showering of more than 100 pustules and increased general erythema of the entire face (Figure 2). Crotamiton cream 10% (every day before noon), ivermectin cream 1% (every night at bedtime), and sodium sulfacetamide cleanser 10% once daily were added to the treatment plan.
Figure 2. Three weeks after starting prednisone, there was a showering of more than 100 pustules and increased general erythema of the entire face due to rosacea fulminans.
At 16 weeks’ gestation, there was slight improvement; however, there was still erythema on the entire face with scattered pustules and multiple papules and nodules. Many small ice-pick scars were seen on the cheeks and forehead. No comedones were observed. A punch biopsy of an intact papule showed a prominent inflammatory infiltrate with granulomatous reaction and numerous neutrophils predominantly affecting hair follicles. Based on the clinical presentation and histopathology, a diagnosis of RF was made. Azithromycin (250 mg once daily) and metronidazole cream 0.75% twice daily were added. Two weeks later there were fewer nodules but many papules, edema, and intense erythema. The prednisone dosage was increased to 40 mg once daily. Two weeks later, the patient showed improvement with fewer lesions, less edema, and less erythema. The patient was instructed to finish the azithromycin course and discontinue use. At 28 weeks’ gestation, a prednisone taper was started with the intention to reduce the daily dose by delivery.
The patient delivered a healthy girl (birth weight, 1.985 kg) prematurely at 34 weeks’ gestation. At 2 months postpartum, the patient’s existing lesions continued to spontaneously improve; however, she still had numerous nodules and papules and continued to develop new lesions and form additional scars. Isotretinoin was instituted at 3 months postpartum upon cessation of nursing. Three months later (40 mg/d isotretinoin), the patient was nearly clear. At 8 months postpartum, isotretinoin was discontinued after a course of 150 mg/kg.
Comment
Rosacea fulminans initially was called pyoderma faciale but was later regarded as a severe form of rosacea and was renamed rosacea fulminans.2 According to a PubMed search of articles indexed for MEDLINE using the terms pregnancy and rosacea fulminans or pyoderma faciale, we identified 12 publications reporting 20 cases of RF associated with pregnancy (Table). Although there is no substantial evidence regarding the exact mechanism, these cases indicate that pregnancy can be an exacerbating or causative factor in the pathogenesis of RF.
In addition to pregnancy, RF has been associated with inflammatory bowel disease, thyroid and liver disease, erythema nodosum, and severe emotional trauma. However, no organism has been consistently isolated, and no evidence of family history has been reported.1 Histopathologic findings are dependent on the stage of disease. Massive infiltrates of neutrophils may be observed in early stages. In older lesions, infiltrates take the form of epithelioid cell granulomas.2
Treatment of RF during pregnancy is challenging. Early and aggressive treatment with retinoids, tetracycline antibiotics, antiandrogenic contraceptives, and dapsone is recommended in patients who are not pregnant; these therapies are all contraindicated in pregnancy. Topical steroids can be safely used; however, systemic steroids usually are required to control RF. The use of systemic steroids can only be justified if the risks for intrauterine growth retardation, maternal diabetes mellitus, and hypertension outweigh the benefits of treating this severe disfiguring skin condition.10 A study by Bakar et al13 indicated that azithromycin is an effective and safe alternative in the treatment of RF. It has a superior pharmacokinetic profile compared to other macrolides and does not pose increased risks for congenital malformation or miscarriage. Because of the concomitant use of both azithromycin and prednisone, it is not possible to determine which had the larger role in the patient’s improvement.
Isotretinoin therapy in our patient led to substantial improvement of RF. Time will tell if the response will be durable. Also unknown is the risk for recurrence with subsequent pregnancies, which has not been reported in the literature. Although it is difficult to confidently say that pregnancy was the inciting factor in this patient’s RF, this case certainly provides more evidence for a link between pregnancy and RF.
Rosacea fulminans (RF) is a rare facial dermatosis characterized by its fulminating course. 1 It presents with superficial and deep-seated papules, pustules, and nodules combined with an intense reddish or cyanotic erythema localized to the face. Furthermore, there is an absence of comedones and involvement of the chest or back. 2 Rosacea fulminans primarily affects women and often is, but not always, proceeded by seborrhea, chronic acne vulgaris, or rosacea. Although the etiology of RF remains unknown, immunologic, hormonal, and vascular factors have been implicated. 3 We report a case of RF in a pregnant patient with a history of mild acne as a teenager that was long ago resolved.
Case Report
A 32-year-old pregnant woman (10 weeks’ gestation) presented with a rapidly progressing inflammatory disorder of the face of 1 month’s duration. The lesions developed 3 weeks after beginning progesterone therapy (200 mg vaginal suppository) for infertility due to polycystic ovary syndrome. Despite discontinuing progesterone for the last month, the patient’s lesions had dramatically worsened (Figure 1). Empiric cephalosporin treatment prescribed by her primary care physician yielded no improvement. Physical examination at the current presentation revealed erythematous nodules and pustules all over the face, coalescing into large thick plaques on the patient’s right cheek and chin. Submental nodes were palpable and tender. Based on the initial clinical findings, acne conglobata secondary to progesterone therapy was considered. The patient was given intralesional triamcinolone (2.5 mg/cc) injections to all larger nodules and several blue light treatments.
Figure 1. Rosacea fulminans in a pregnant woman at presentation (10 weeks’ gestation).
The injected areas had improved 5 days after the initial visit; however, the chin and right paranasal cheek developed even more nodules and papules coalescing into large plaques. After consulting the patient’s obstetrician, prednisone (20 mg once daily) was initiated. Three weeks later, the patient’s nodular lesions had improved, but there was a showering of more than 100 pustules and increased general erythema of the entire face (Figure 2). Crotamiton cream 10% (every day before noon), ivermectin cream 1% (every night at bedtime), and sodium sulfacetamide cleanser 10% once daily were added to the treatment plan.
Figure 2. Three weeks after starting prednisone, there was a showering of more than 100 pustules and increased general erythema of the entire face due to rosacea fulminans.
At 16 weeks’ gestation, there was slight improvement; however, there was still erythema on the entire face with scattered pustules and multiple papules and nodules. Many small ice-pick scars were seen on the cheeks and forehead. No comedones were observed. A punch biopsy of an intact papule showed a prominent inflammatory infiltrate with granulomatous reaction and numerous neutrophils predominantly affecting hair follicles. Based on the clinical presentation and histopathology, a diagnosis of RF was made. Azithromycin (250 mg once daily) and metronidazole cream 0.75% twice daily were added. Two weeks later there were fewer nodules but many papules, edema, and intense erythema. The prednisone dosage was increased to 40 mg once daily. Two weeks later, the patient showed improvement with fewer lesions, less edema, and less erythema. The patient was instructed to finish the azithromycin course and discontinue use. At 28 weeks’ gestation, a prednisone taper was started with the intention to reduce the daily dose by delivery.
The patient delivered a healthy girl (birth weight, 1.985 kg) prematurely at 34 weeks’ gestation. At 2 months postpartum, the patient’s existing lesions continued to spontaneously improve; however, she still had numerous nodules and papules and continued to develop new lesions and form additional scars. Isotretinoin was instituted at 3 months postpartum upon cessation of nursing. Three months later (40 mg/d isotretinoin), the patient was nearly clear. At 8 months postpartum, isotretinoin was discontinued after a course of 150 mg/kg.
Comment
Rosacea fulminans initially was called pyoderma faciale but was later regarded as a severe form of rosacea and was renamed rosacea fulminans.2 According to a PubMed search of articles indexed for MEDLINE using the terms pregnancy and rosacea fulminans or pyoderma faciale, we identified 12 publications reporting 20 cases of RF associated with pregnancy (Table). Although there is no substantial evidence regarding the exact mechanism, these cases indicate that pregnancy can be an exacerbating or causative factor in the pathogenesis of RF.
In addition to pregnancy, RF has been associated with inflammatory bowel disease, thyroid and liver disease, erythema nodosum, and severe emotional trauma. However, no organism has been consistently isolated, and no evidence of family history has been reported.1 Histopathologic findings are dependent on the stage of disease. Massive infiltrates of neutrophils may be observed in early stages. In older lesions, infiltrates take the form of epithelioid cell granulomas.2
Treatment of RF during pregnancy is challenging. Early and aggressive treatment with retinoids, tetracycline antibiotics, antiandrogenic contraceptives, and dapsone is recommended in patients who are not pregnant; these therapies are all contraindicated in pregnancy. Topical steroids can be safely used; however, systemic steroids usually are required to control RF. The use of systemic steroids can only be justified if the risks for intrauterine growth retardation, maternal diabetes mellitus, and hypertension outweigh the benefits of treating this severe disfiguring skin condition.10 A study by Bakar et al13 indicated that azithromycin is an effective and safe alternative in the treatment of RF. It has a superior pharmacokinetic profile compared to other macrolides and does not pose increased risks for congenital malformation or miscarriage. Because of the concomitant use of both azithromycin and prednisone, it is not possible to determine which had the larger role in the patient’s improvement.
Isotretinoin therapy in our patient led to substantial improvement of RF. Time will tell if the response will be durable. Also unknown is the risk for recurrence with subsequent pregnancies, which has not been reported in the literature. Although it is difficult to confidently say that pregnancy was the inciting factor in this patient’s RF, this case certainly provides more evidence for a link between pregnancy and RF.
References
Jarrett R, Gonsalves R, Anstey AV. Differing obstetric outcomes of rosacea fulminans in pregnancy: report of three cases with review of pathogenesis and management. Clin Exp Dermatol. 2010;35:888-891. doi:10.1111/j.1365-2230.2010.03846.x
Ferahbas A, Utas S, Mistik S, et al. Rosacea fulminans in pregnancy: case report and review of the literature. Am J Clin Dermatol. 2006;7:141-144. doi:10.2165/00128071-200607020-00007
Fuentelsaz V, Ara M, Corredera C, et al. Rosacea fulminans in pregnancy: successful treatment with azithromycin. Clin Exp Dermatol. 2011;36:674-676. doi:10.1111/j.1365-2230.2011.04042.x
Garayar Cantero M, Garabito Solovera E, Aguado García Á, et al. Use of permethrin in the treatment of rosacea fulminans during pregnancy: one case report. Dermatol Ther. 2020;33:E13436. doi:10.1111/dth.13436
Demir O, Tas IS, Gunay B, et al. A rare dermatologic disease in pregnancy: rosacea fulminans—case report and review of the literature. Open Access Maced J Med Sci. 2018;6:1438-1441. doi:10.3889/oamjms.2018.267
Markou AG, Alessandrini V, Muray JM, et al. Rosacea fulminans during pregnancy. Clin Exp Obstet Gynecol. 2017;44:157-159.
Haenen CCP, Kouwenhoven STP, van Doorn R. Rosacea fulminans in pregnancy [in Dutch]. Ned Tijdschr Geneeskd. 2015;159:A8334.
de Morais e Silva FA, Bonassi M, Steiner D, et al. Rosacea fulminans in pregnancy with ocular perforation. J Dtsch Dermatol Ges. 2011;9:542-543. doi:10.1111/j.1610-0387.2011.07616.x
Cisse M, Maruani A, Bré C. Rosacea fulminans in the early course of a pregnancy by in vitro fertilization with embryo transfer [in French]. Ann Dermatol Venereol. 2008;135:675-678. doi:10.1016/j.annder.2008.04.015
Lewis VJ, Holme SA, Wright A, et al. Rosacea fulminans in pregnancy. Br J Dermatol. 2004;151:917-919. doi:10.1111/j.1365-2133.2004.06190.x
Plewig G, Jansen T, Kligman AM. Pyoderma faciale. a review and report of 20 additional cases: is it rosacea? Arch Dermatol. 1992;128:1611-1617. doi:10.1001/archderm.128.12.1611
Massa MC, Su WP. Pyoderma faciale: a clinical study of twenty-nine patients. J Am Acad Dermatol. 1982;6:84-91. doi:10.1016/s0190-9622(82)70008-8
Bakar O, Demirçay Z, Gürbüz O. Therapeutic potential of azithromycin in rosacea. Int J Dermatol. 2004;43:151-154. doi:10.1111/j.1365-4632.2004.01958.x
References
Jarrett R, Gonsalves R, Anstey AV. Differing obstetric outcomes of rosacea fulminans in pregnancy: report of three cases with review of pathogenesis and management. Clin Exp Dermatol. 2010;35:888-891. doi:10.1111/j.1365-2230.2010.03846.x
Ferahbas A, Utas S, Mistik S, et al. Rosacea fulminans in pregnancy: case report and review of the literature. Am J Clin Dermatol. 2006;7:141-144. doi:10.2165/00128071-200607020-00007
Fuentelsaz V, Ara M, Corredera C, et al. Rosacea fulminans in pregnancy: successful treatment with azithromycin. Clin Exp Dermatol. 2011;36:674-676. doi:10.1111/j.1365-2230.2011.04042.x
Garayar Cantero M, Garabito Solovera E, Aguado García Á, et al. Use of permethrin in the treatment of rosacea fulminans during pregnancy: one case report. Dermatol Ther. 2020;33:E13436. doi:10.1111/dth.13436
Demir O, Tas IS, Gunay B, et al. A rare dermatologic disease in pregnancy: rosacea fulminans—case report and review of the literature. Open Access Maced J Med Sci. 2018;6:1438-1441. doi:10.3889/oamjms.2018.267
Markou AG, Alessandrini V, Muray JM, et al. Rosacea fulminans during pregnancy. Clin Exp Obstet Gynecol. 2017;44:157-159.
Haenen CCP, Kouwenhoven STP, van Doorn R. Rosacea fulminans in pregnancy [in Dutch]. Ned Tijdschr Geneeskd. 2015;159:A8334.
de Morais e Silva FA, Bonassi M, Steiner D, et al. Rosacea fulminans in pregnancy with ocular perforation. J Dtsch Dermatol Ges. 2011;9:542-543. doi:10.1111/j.1610-0387.2011.07616.x
Cisse M, Maruani A, Bré C. Rosacea fulminans in the early course of a pregnancy by in vitro fertilization with embryo transfer [in French]. Ann Dermatol Venereol. 2008;135:675-678. doi:10.1016/j.annder.2008.04.015
Lewis VJ, Holme SA, Wright A, et al. Rosacea fulminans in pregnancy. Br J Dermatol. 2004;151:917-919. doi:10.1111/j.1365-2133.2004.06190.x
Plewig G, Jansen T, Kligman AM. Pyoderma faciale. a review and report of 20 additional cases: is it rosacea? Arch Dermatol. 1992;128:1611-1617. doi:10.1001/archderm.128.12.1611
Massa MC, Su WP. Pyoderma faciale: a clinical study of twenty-nine patients. J Am Acad Dermatol. 1982;6:84-91. doi:10.1016/s0190-9622(82)70008-8
Bakar O, Demirçay Z, Gürbüz O. Therapeutic potential of azithromycin in rosacea. Int J Dermatol. 2004;43:151-154. doi:10.1111/j.1365-4632.2004.01958.x
Perioperative anxiety is common in patients undergoing nail surgery. Patients might worry about seeing blood; about the procedure itself, including nail avulsion; and about associated pain and disfigurement. Nail surgery causes a high level of anxiety that correlates positively with postoperative pain1 and overall patient dissatisfaction. Furthermore, surgery-related anxiety is a predictor of increased postoperative analgesic use2 and delayed recovery.3
Therefore, implementing strategies that reduce perioperative anxiety may help minimize postoperative pain. Squeezing a stress ball, hand-holding, virtual reality, and music are tools that have been studied to reduce anxiety in the context of Mohs micrographic surgery; these strategies have not been studied for nail surgery.
The Technique
Using a sleep mask is a practical solution to reduce patient anxiety during nail surgery. A minority of patients will choose to watch their surgical procedure; most become unnerved observing their nail surgery. Using a sleep mask diverts visual attention from the surgical field without physically interfering with the nail surgeon. Utilizing a sleep mask is cost-effective, with disposable sleep masks available online for less than $0.30 each. Patients can bring their own mask, or a mask can be offered prior to surgery.
If desired, patients are instructed to wear the sleep mask during the entirety of the procedure, starting from anesthetic infiltration until wound closure and dressing application. Any adjustments can be made with the patient’s free hand. The sleep mask can be offered to patients of all ages undergoing nail surgery under local anesthesia, except babies and young children, who require general anesthesia.
Practical Implications
Distraction is an important strategy to reduce anxiety and pain in patients undergoing surgical procedures. In an observational study of 3087 surgical patients, 36% reported that self-distraction was the most helpful strategy for coping with preoperative anxiety.4 In a randomized, open-label clinical trial of 72 patients undergoing peripheral venous catheterization, asking the patients simple questions during the procedure was more effective than local anesthesia in reducing the perception of pain.5
It is crucial to implement strategies to reduce anxiety in patients undergoing nail surgery. Using a sleep mask impedes direct visualization of the surgical field, thus distracting the patient’s sight and attention from the procedure. Furthermore, this technique is safe and cost-effective.
Controlled clinical trials are necessary to assess the efficacy of this method in reducing nail surgery–related anxiety in comparison to other techniques.
References
Navarro-Gastón D, Munuera-Martínez PV. Prevalence of preoperative anxiety and its relationship with postoperative pain in foot nail surgery: a cross-sectional study. Int J Environ Res Public Health. 2020;17:4481. doi:10.3390/ijerph17124481
Ip HYV, Abrishami A, Peng PWH, et al. Predictors of postoperative pain and analgesic consumption: a qualitative systematic review. Anesthesiology. 2009;111:657-677. doi:10.1097/ALN.0b013e3181aae87a
Mavros MN, Athanasiou S, Gkegkes ID, et al. Do psychological variables affect early surgical recovery? PLoS One. 2011;6:E20306. doi:10.1371/journal.pone.0020306
Aust H, Rüsch D, Schuster M, et al. Coping strategies in anxious surgical patients. BMC Health Serv Res. 2016;16:250. doi:10.1186/s12913-016-1492-5
Balanyuk I, Ledonne G, Provenzano M, et al. Distraction technique for pain reduction in peripheral venous catheterization: randomized, controlled trial. Acta Biomed. 2018;89(suppl 4):55-63. doi:10.23750/abmv89i4-S.7115
Perioperative anxiety is common in patients undergoing nail surgery. Patients might worry about seeing blood; about the procedure itself, including nail avulsion; and about associated pain and disfigurement. Nail surgery causes a high level of anxiety that correlates positively with postoperative pain1 and overall patient dissatisfaction. Furthermore, surgery-related anxiety is a predictor of increased postoperative analgesic use2 and delayed recovery.3
Therefore, implementing strategies that reduce perioperative anxiety may help minimize postoperative pain. Squeezing a stress ball, hand-holding, virtual reality, and music are tools that have been studied to reduce anxiety in the context of Mohs micrographic surgery; these strategies have not been studied for nail surgery.
The Technique
Using a sleep mask is a practical solution to reduce patient anxiety during nail surgery. A minority of patients will choose to watch their surgical procedure; most become unnerved observing their nail surgery. Using a sleep mask diverts visual attention from the surgical field without physically interfering with the nail surgeon. Utilizing a sleep mask is cost-effective, with disposable sleep masks available online for less than $0.30 each. Patients can bring their own mask, or a mask can be offered prior to surgery.
If desired, patients are instructed to wear the sleep mask during the entirety of the procedure, starting from anesthetic infiltration until wound closure and dressing application. Any adjustments can be made with the patient’s free hand. The sleep mask can be offered to patients of all ages undergoing nail surgery under local anesthesia, except babies and young children, who require general anesthesia.
Practical Implications
Distraction is an important strategy to reduce anxiety and pain in patients undergoing surgical procedures. In an observational study of 3087 surgical patients, 36% reported that self-distraction was the most helpful strategy for coping with preoperative anxiety.4 In a randomized, open-label clinical trial of 72 patients undergoing peripheral venous catheterization, asking the patients simple questions during the procedure was more effective than local anesthesia in reducing the perception of pain.5
It is crucial to implement strategies to reduce anxiety in patients undergoing nail surgery. Using a sleep mask impedes direct visualization of the surgical field, thus distracting the patient’s sight and attention from the procedure. Furthermore, this technique is safe and cost-effective.
Controlled clinical trials are necessary to assess the efficacy of this method in reducing nail surgery–related anxiety in comparison to other techniques.
Practice Gap
Perioperative anxiety is common in patients undergoing nail surgery. Patients might worry about seeing blood; about the procedure itself, including nail avulsion; and about associated pain and disfigurement. Nail surgery causes a high level of anxiety that correlates positively with postoperative pain1 and overall patient dissatisfaction. Furthermore, surgery-related anxiety is a predictor of increased postoperative analgesic use2 and delayed recovery.3
Therefore, implementing strategies that reduce perioperative anxiety may help minimize postoperative pain. Squeezing a stress ball, hand-holding, virtual reality, and music are tools that have been studied to reduce anxiety in the context of Mohs micrographic surgery; these strategies have not been studied for nail surgery.
The Technique
Using a sleep mask is a practical solution to reduce patient anxiety during nail surgery. A minority of patients will choose to watch their surgical procedure; most become unnerved observing their nail surgery. Using a sleep mask diverts visual attention from the surgical field without physically interfering with the nail surgeon. Utilizing a sleep mask is cost-effective, with disposable sleep masks available online for less than $0.30 each. Patients can bring their own mask, or a mask can be offered prior to surgery.
If desired, patients are instructed to wear the sleep mask during the entirety of the procedure, starting from anesthetic infiltration until wound closure and dressing application. Any adjustments can be made with the patient’s free hand. The sleep mask can be offered to patients of all ages undergoing nail surgery under local anesthesia, except babies and young children, who require general anesthesia.
Practical Implications
Distraction is an important strategy to reduce anxiety and pain in patients undergoing surgical procedures. In an observational study of 3087 surgical patients, 36% reported that self-distraction was the most helpful strategy for coping with preoperative anxiety.4 In a randomized, open-label clinical trial of 72 patients undergoing peripheral venous catheterization, asking the patients simple questions during the procedure was more effective than local anesthesia in reducing the perception of pain.5
It is crucial to implement strategies to reduce anxiety in patients undergoing nail surgery. Using a sleep mask impedes direct visualization of the surgical field, thus distracting the patient’s sight and attention from the procedure. Furthermore, this technique is safe and cost-effective.
Controlled clinical trials are necessary to assess the efficacy of this method in reducing nail surgery–related anxiety in comparison to other techniques.
References
Navarro-Gastón D, Munuera-Martínez PV. Prevalence of preoperative anxiety and its relationship with postoperative pain in foot nail surgery: a cross-sectional study. Int J Environ Res Public Health. 2020;17:4481. doi:10.3390/ijerph17124481
Ip HYV, Abrishami A, Peng PWH, et al. Predictors of postoperative pain and analgesic consumption: a qualitative systematic review. Anesthesiology. 2009;111:657-677. doi:10.1097/ALN.0b013e3181aae87a
Mavros MN, Athanasiou S, Gkegkes ID, et al. Do psychological variables affect early surgical recovery? PLoS One. 2011;6:E20306. doi:10.1371/journal.pone.0020306
Aust H, Rüsch D, Schuster M, et al. Coping strategies in anxious surgical patients. BMC Health Serv Res. 2016;16:250. doi:10.1186/s12913-016-1492-5
Balanyuk I, Ledonne G, Provenzano M, et al. Distraction technique for pain reduction in peripheral venous catheterization: randomized, controlled trial. Acta Biomed. 2018;89(suppl 4):55-63. doi:10.23750/abmv89i4-S.7115
References
Navarro-Gastón D, Munuera-Martínez PV. Prevalence of preoperative anxiety and its relationship with postoperative pain in foot nail surgery: a cross-sectional study. Int J Environ Res Public Health. 2020;17:4481. doi:10.3390/ijerph17124481
Ip HYV, Abrishami A, Peng PWH, et al. Predictors of postoperative pain and analgesic consumption: a qualitative systematic review. Anesthesiology. 2009;111:657-677. doi:10.1097/ALN.0b013e3181aae87a
Mavros MN, Athanasiou S, Gkegkes ID, et al. Do psychological variables affect early surgical recovery? PLoS One. 2011;6:E20306. doi:10.1371/journal.pone.0020306
Aust H, Rüsch D, Schuster M, et al. Coping strategies in anxious surgical patients. BMC Health Serv Res. 2016;16:250. doi:10.1186/s12913-016-1492-5
Balanyuk I, Ledonne G, Provenzano M, et al. Distraction technique for pain reduction in peripheral venous catheterization: randomized, controlled trial. Acta Biomed. 2018;89(suppl 4):55-63. doi:10.23750/abmv89i4-S.7115
Congenital onychodysplasia of the index finger (COIF), or Iso-Kikuchi syndrome, is a rare disorder characterized by malformation of one or both nails of the index fingers. The various anomalies described are anonychia, micronychia, polyonychia, malalignment, or hemi-onychogryphosis. It may be associated with abnormalities of the underlying phalangeal bone, the most masked being bifurcation of the terminal phalange.1 Initially thought to be nonhereditary and nonfamilial,2 it is now known that COIF can be inherited in an autosomal-dominant fashion.3 Millman and Strier3 described a family of 9 patients with COIF. It rarely is described outside of Japan. Padmavathy et al4 described a case in an Indian patient with COIF that was associated with the absence of a ring finger in addition to anomalies of the metacarpal bones.
Congenital onychodysplasia of the index finger has a broad spectrum regarding its etiology and clinical features.5 The pathogenesis of COIF still is poorly understood. Deficient circulation in digital arteries is thought to be a putative mechanism for developing a deformed nail. The nail is affected on the radial side of the index finger, likely because of the smaller caliber of the artery on that side.5 Hereditary as well as nonhereditary sporadic cases have been reported. In addition to the various fingernail anomalies, skeletal abnormalities also have been reported. Baran and Stroud6 have reported deformed lunulae as a manifestation of COIF.
The Diagnosis: Congenital Onychodysplasia of the Index Finger
The differential diagnosis of COIF includes hidrotic ectodermal dysplasia, nail-patella syndrome, Poland syndrome, and DOOR syndrome. Hidrotic ectodermal dysplasia exhibits onychodystrophy, generalized hypotrichosis, palmoplantar keratoderma, and dental anomalies.7 Nail-patella syndrome presents with hypoplasia of the fingernails and toenails, triangular nail lunulae, absent or hypoplastic patellae, and elbow and iliac horn dysplasia. Poland syndrome is distinguished from COIF by the congenital absence of the pectoralis major muscle on the ipsilateral side of the involved digits. The DOOR syndrome tetrad is comprised of deafness, onychodystrophy, osteodystrophy, and mental retardation.8 Unlike these conditions, COIF does not involve systems other than the nails and phalanges.
Treatment of this condition is mainly conservative, as patients typically do not have symptoms.9 Surgical interventions can be considered for cosmetic concerns. Knowledge of this congenital entity and its clinical findings is essential to prevent unnecessary procedures and workup.
References
De Berker AR, Baran R. Science of the nail apparatus. Diseases of the Nails and Their Management. In: Baran R, De Berker AR, Holzberg M, et al, eds. 4th ed. Willey-Blackwell; 2012:1-50.
Kikuchi I, Horikawa S, Amano F. Congenital onychodysplasia of the index fingers. Arch Dermatol. 1974;110:743-746.
Millman AJ, Strier RP. Congenital onychodysplasia of the index fingers: report of a family. J Am Acad Dermatol. 1982;7:57-65.
Padmavathy L, Rao L, Ethirajan N, et al. Iso-Kikuchi syndrome with absence of ring fingers and metacarpal bone abnormality. Indian J Dermatol Venereol Leprol. 2008;74:513.
Hadj-Rabia S, Juhlin L, Baran R. Hereditary and congenital nail disorders. In: Baran R, De Berker AR, Holzberg M, et al, eds. Diseases of the Nails and Their Management. 4th ed. Wiley-Blackwell; 2012:485-490.
Baran R, Stroud JD. Congenital onychodysplasia of the index fingers: Iso and Kikuchi syndrome. Arch Dermatol. 1984;120:243-244.
Valerio E, Favot F, Mattei I, et al. Congenital isolated Iso-Kikuchi syndrome in a newborn. Clin Case Rep. 2015;3:866.
Danarti R, Rahmayani S, Wirohadidjojo YW, et al. Deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures (DOORS) syndrome: a new case report from Indonesia and review of the literature. Eur J Dermatol. 2020;30:404-407.
Milani-Nejad N, Mosser-Goldfarb J. Congenital onychodysplasia of index fingers: Iso-Kikuchi syndrome. J Pediatr. 2020;218:254.
Congenital onychodysplasia of the index finger (COIF), or Iso-Kikuchi syndrome, is a rare disorder characterized by malformation of one or both nails of the index fingers. The various anomalies described are anonychia, micronychia, polyonychia, malalignment, or hemi-onychogryphosis. It may be associated with abnormalities of the underlying phalangeal bone, the most masked being bifurcation of the terminal phalange.1 Initially thought to be nonhereditary and nonfamilial,2 it is now known that COIF can be inherited in an autosomal-dominant fashion.3 Millman and Strier3 described a family of 9 patients with COIF. It rarely is described outside of Japan. Padmavathy et al4 described a case in an Indian patient with COIF that was associated with the absence of a ring finger in addition to anomalies of the metacarpal bones.
Congenital onychodysplasia of the index finger has a broad spectrum regarding its etiology and clinical features.5 The pathogenesis of COIF still is poorly understood. Deficient circulation in digital arteries is thought to be a putative mechanism for developing a deformed nail. The nail is affected on the radial side of the index finger, likely because of the smaller caliber of the artery on that side.5 Hereditary as well as nonhereditary sporadic cases have been reported. In addition to the various fingernail anomalies, skeletal abnormalities also have been reported. Baran and Stroud6 have reported deformed lunulae as a manifestation of COIF.
The Diagnosis: Congenital Onychodysplasia of the Index Finger
The differential diagnosis of COIF includes hidrotic ectodermal dysplasia, nail-patella syndrome, Poland syndrome, and DOOR syndrome. Hidrotic ectodermal dysplasia exhibits onychodystrophy, generalized hypotrichosis, palmoplantar keratoderma, and dental anomalies.7 Nail-patella syndrome presents with hypoplasia of the fingernails and toenails, triangular nail lunulae, absent or hypoplastic patellae, and elbow and iliac horn dysplasia. Poland syndrome is distinguished from COIF by the congenital absence of the pectoralis major muscle on the ipsilateral side of the involved digits. The DOOR syndrome tetrad is comprised of deafness, onychodystrophy, osteodystrophy, and mental retardation.8 Unlike these conditions, COIF does not involve systems other than the nails and phalanges.
Treatment of this condition is mainly conservative, as patients typically do not have symptoms.9 Surgical interventions can be considered for cosmetic concerns. Knowledge of this congenital entity and its clinical findings is essential to prevent unnecessary procedures and workup.
Congenital onychodysplasia of the index finger (COIF), or Iso-Kikuchi syndrome, is a rare disorder characterized by malformation of one or both nails of the index fingers. The various anomalies described are anonychia, micronychia, polyonychia, malalignment, or hemi-onychogryphosis. It may be associated with abnormalities of the underlying phalangeal bone, the most masked being bifurcation of the terminal phalange.1 Initially thought to be nonhereditary and nonfamilial,2 it is now known that COIF can be inherited in an autosomal-dominant fashion.3 Millman and Strier3 described a family of 9 patients with COIF. It rarely is described outside of Japan. Padmavathy et al4 described a case in an Indian patient with COIF that was associated with the absence of a ring finger in addition to anomalies of the metacarpal bones.
Congenital onychodysplasia of the index finger has a broad spectrum regarding its etiology and clinical features.5 The pathogenesis of COIF still is poorly understood. Deficient circulation in digital arteries is thought to be a putative mechanism for developing a deformed nail. The nail is affected on the radial side of the index finger, likely because of the smaller caliber of the artery on that side.5 Hereditary as well as nonhereditary sporadic cases have been reported. In addition to the various fingernail anomalies, skeletal abnormalities also have been reported. Baran and Stroud6 have reported deformed lunulae as a manifestation of COIF.
The Diagnosis: Congenital Onychodysplasia of the Index Finger
The differential diagnosis of COIF includes hidrotic ectodermal dysplasia, nail-patella syndrome, Poland syndrome, and DOOR syndrome. Hidrotic ectodermal dysplasia exhibits onychodystrophy, generalized hypotrichosis, palmoplantar keratoderma, and dental anomalies.7 Nail-patella syndrome presents with hypoplasia of the fingernails and toenails, triangular nail lunulae, absent or hypoplastic patellae, and elbow and iliac horn dysplasia. Poland syndrome is distinguished from COIF by the congenital absence of the pectoralis major muscle on the ipsilateral side of the involved digits. The DOOR syndrome tetrad is comprised of deafness, onychodystrophy, osteodystrophy, and mental retardation.8 Unlike these conditions, COIF does not involve systems other than the nails and phalanges.
Treatment of this condition is mainly conservative, as patients typically do not have symptoms.9 Surgical interventions can be considered for cosmetic concerns. Knowledge of this congenital entity and its clinical findings is essential to prevent unnecessary procedures and workup.
References
De Berker AR, Baran R. Science of the nail apparatus. Diseases of the Nails and Their Management. In: Baran R, De Berker AR, Holzberg M, et al, eds. 4th ed. Willey-Blackwell; 2012:1-50.
Kikuchi I, Horikawa S, Amano F. Congenital onychodysplasia of the index fingers. Arch Dermatol. 1974;110:743-746.
Millman AJ, Strier RP. Congenital onychodysplasia of the index fingers: report of a family. J Am Acad Dermatol. 1982;7:57-65.
Padmavathy L, Rao L, Ethirajan N, et al. Iso-Kikuchi syndrome with absence of ring fingers and metacarpal bone abnormality. Indian J Dermatol Venereol Leprol. 2008;74:513.
Hadj-Rabia S, Juhlin L, Baran R. Hereditary and congenital nail disorders. In: Baran R, De Berker AR, Holzberg M, et al, eds. Diseases of the Nails and Their Management. 4th ed. Wiley-Blackwell; 2012:485-490.
Baran R, Stroud JD. Congenital onychodysplasia of the index fingers: Iso and Kikuchi syndrome. Arch Dermatol. 1984;120:243-244.
Valerio E, Favot F, Mattei I, et al. Congenital isolated Iso-Kikuchi syndrome in a newborn. Clin Case Rep. 2015;3:866.
Danarti R, Rahmayani S, Wirohadidjojo YW, et al. Deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures (DOORS) syndrome: a new case report from Indonesia and review of the literature. Eur J Dermatol. 2020;30:404-407.
Milani-Nejad N, Mosser-Goldfarb J. Congenital onychodysplasia of index fingers: Iso-Kikuchi syndrome. J Pediatr. 2020;218:254.
References
De Berker AR, Baran R. Science of the nail apparatus. Diseases of the Nails and Their Management. In: Baran R, De Berker AR, Holzberg M, et al, eds. 4th ed. Willey-Blackwell; 2012:1-50.
Kikuchi I, Horikawa S, Amano F. Congenital onychodysplasia of the index fingers. Arch Dermatol. 1974;110:743-746.
Millman AJ, Strier RP. Congenital onychodysplasia of the index fingers: report of a family. J Am Acad Dermatol. 1982;7:57-65.
Padmavathy L, Rao L, Ethirajan N, et al. Iso-Kikuchi syndrome with absence of ring fingers and metacarpal bone abnormality. Indian J Dermatol Venereol Leprol. 2008;74:513.
Hadj-Rabia S, Juhlin L, Baran R. Hereditary and congenital nail disorders. In: Baran R, De Berker AR, Holzberg M, et al, eds. Diseases of the Nails and Their Management. 4th ed. Wiley-Blackwell; 2012:485-490.
Baran R, Stroud JD. Congenital onychodysplasia of the index fingers: Iso and Kikuchi syndrome. Arch Dermatol. 1984;120:243-244.
Valerio E, Favot F, Mattei I, et al. Congenital isolated Iso-Kikuchi syndrome in a newborn. Clin Case Rep. 2015;3:866.
Danarti R, Rahmayani S, Wirohadidjojo YW, et al. Deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures (DOORS) syndrome: a new case report from Indonesia and review of the literature. Eur J Dermatol. 2020;30:404-407.
Milani-Nejad N, Mosser-Goldfarb J. Congenital onychodysplasia of index fingers: Iso-Kikuchi syndrome. J Pediatr. 2020;218:254.
A 21-year-old Indian woman who was initially seeking dermatology consultation for acne also was noted to have micronychia of the nail of the left index finger. The affected nail was narrow and half as broad as the unaffected normal nail on the right index finger. The patient confirmed that this finding had been present since birth; she faced no cosmetic disability and had not sought medical care for diagnosis or treatment. There was no history of trauma, complications during pregnancy, family history of micronychia or similar eruptions, or any other inciting event. The teeth, hair, and skin as well as the patient’s height, weight, and physical and mental development were normal. Systemic examination revealed no abnormalities. Radiography of the hands did not reveal any apparent bony abnormalities.
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