The Journal of Family Practice

References

1. CDC. Coronavirus Disease 2019 (COVID-19): Cases in the US. www.cdc.gov/coronavirus/2019-ncov/cases-updates/cases-in-us.html. Accessed August 18, 2020.
2. US Department of Health and Human Services. Fact Sheet: explaining Operation Warp Speed. www.hhs.gov/coronavirus/explaining-operation-warp-speed/index.html. Accessed August 18, 2020.
3. O’Callahan KP, Blatz AM, Offit PA. Developing a SARS-CoV-2 vaccine at warp speed. JAMA. 2020;324:437-438.
4. Pardi N, Hogan MJ, Porter FW, et al. mRNA vaccines—a new era in vaccinology. Nat Rev Drug Discov. 2018;17:261-279.
5. Lurie N, Sharfstein JM, Goodman JL. The development of COVID-19 vaccines: safeguards needed [commentary]. JAMA. 2020;324:439-440.
6. Salman DA, Akhtar A, Mergler MJ, et al; H1N1 Working Group of Federal Immunization Safety Task Force. Immunization safety monitoring systems for the 2009 H1N1 monovalent influenza vaccination program. Pediatrics. 2011;127(suppl 1):S78-S86.

References

1. CDC. Coronavirus Disease 2019 (COVID-19): Cases in the US. www.cdc.gov/coronavirus/2019-ncov/cases-updates/cases-in-us.html. Accessed August 18, 2020.
2. US Department of Health and Human Services. Fact Sheet: explaining Operation Warp Speed. www.hhs.gov/coronavirus/explaining-operation-warp-speed/index.html. Accessed August 18, 2020.
3. O’Callahan KP, Blatz AM, Offit PA. Developing a SARS-CoV-2 vaccine at warp speed. JAMA. 2020;324:437-438.
4. Pardi N, Hogan MJ, Porter FW, et al. mRNA vaccines—a new era in vaccinology. Nat Rev Drug Discov. 2018;17:261-279.
5. Lurie N, Sharfstein JM, Goodman JL. The development of COVID-19 vaccines: safeguards needed [commentary]. JAMA. 2020;324:439-440.
6. Salman DA, Akhtar A, Mergler MJ, et al; H1N1 Working Group of Federal Immunization Safety Task Force. Immunization safety monitoring systems for the 2009 H1N1 monovalent influenza vaccination program. Pediatrics. 2011;127(suppl 1):S78-S86.

References

1. CDC. Coronavirus Disease 2019 (COVID-19): Cases in the US. www.cdc.gov/coronavirus/2019-ncov/cases-updates/cases-in-us.html. Accessed August 18, 2020.
2. US Department of Health and Human Services. Fact Sheet: explaining Operation Warp Speed. www.hhs.gov/coronavirus/explaining-operation-warp-speed/index.html. Accessed August 18, 2020.
3. O’Callahan KP, Blatz AM, Offit PA. Developing a SARS-CoV-2 vaccine at warp speed. JAMA. 2020;324:437-438.
4. Pardi N, Hogan MJ, Porter FW, et al. mRNA vaccines—a new era in vaccinology. Nat Rev Drug Discov. 2018;17:261-279.
5. Lurie N, Sharfstein JM, Goodman JL. The development of COVID-19 vaccines: safeguards needed [commentary]. JAMA. 2020;324:439-440.
6. Salman DA, Akhtar A, Mergler MJ, et al; H1N1 Working Group of Federal Immunization Safety Task Force. Immunization safety monitoring systems for the 2009 H1N1 monovalent influenza vaccination program. Pediatrics. 2011;127(suppl 1):S78-S86.

The photograph submitted for the telemedicine visit showed 2 classic umbilicated lesions and 1 dome-shaped papule consistent with molluscum contagiosum. Not all skin conditions can be diagnosed or treated via telehealth, but with a careful history, cooperative patients (and parents in this case), and photos taken on newer cell phones or digital cameras, many conditions can be diagnosed and managed appropriately.

Molluscum contagiosum is caused by the Molluscipox genus poxvirus and Is commonly seen in preschool and school-aged children. It can be passed through direct contact with infected individuals or spread by fomites. (In this case, the child may have picked up the virus by sharing a towel with an infected individual.)

The flesh-colored lesions are umbilicated or popular, and occur in clusters on the trunk, face, and extremities. Typically, the lesions will resolve spontaneously, but it may take several weeks to many months for resolution.

Given this lengthy time for spontaneous resolution, the risk of spreading to family members or other contacts, and the skin’s appearance, many patients choose to treat the lesions. Treatment options include curettage, cryosurgery, and laser. Available topical destructive agents include podophyllotoxin, trichloroacetic acid, benzoyl peroxide, potassium hydroxide, and cantharidin (which is from the blister beetle and often difficult to obtain). There also are naturopathic topical products and immune system modulators, including topical imiquimod. These treatments are commonly used, but are off-label for the treatment of molluscum contagiosum.

The family was counseled that there is debate about the effectiveness of imiquimod for molluscum contagiosum, but that some studies find it to be useful. In this case, the mother chose a prescription for imiquimod cream 5%, to be applied 3 times weekly at bedtime until the lesions resolved. (The cream can be used for up to 16 weeks.) The family was advised that erythema and irritation are expected adverse effects at the application site.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

The photograph submitted for the telemedicine visit showed 2 classic umbilicated lesions and 1 dome-shaped papule consistent with molluscum contagiosum. Not all skin conditions can be diagnosed or treated via telehealth, but with a careful history, cooperative patients (and parents in this case), and photos taken on newer cell phones or digital cameras, many conditions can be diagnosed and managed appropriately.

Molluscum contagiosum is caused by the Molluscipox genus poxvirus and Is commonly seen in preschool and school-aged children. It can be passed through direct contact with infected individuals or spread by fomites. (In this case, the child may have picked up the virus by sharing a towel with an infected individual.)

The flesh-colored lesions are umbilicated or popular, and occur in clusters on the trunk, face, and extremities. Typically, the lesions will resolve spontaneously, but it may take several weeks to many months for resolution.

Given this lengthy time for spontaneous resolution, the risk of spreading to family members or other contacts, and the skin’s appearance, many patients choose to treat the lesions. Treatment options include curettage, cryosurgery, and laser. Available topical destructive agents include podophyllotoxin, trichloroacetic acid, benzoyl peroxide, potassium hydroxide, and cantharidin (which is from the blister beetle and often difficult to obtain). There also are naturopathic topical products and immune system modulators, including topical imiquimod. These treatments are commonly used, but are off-label for the treatment of molluscum contagiosum.

The family was counseled that there is debate about the effectiveness of imiquimod for molluscum contagiosum, but that some studies find it to be useful. In this case, the mother chose a prescription for imiquimod cream 5%, to be applied 3 times weekly at bedtime until the lesions resolved. (The cream can be used for up to 16 weeks.) The family was advised that erythema and irritation are expected adverse effects at the application site.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

The photograph submitted for the telemedicine visit showed 2 classic umbilicated lesions and 1 dome-shaped papule consistent with molluscum contagiosum. Not all skin conditions can be diagnosed or treated via telehealth, but with a careful history, cooperative patients (and parents in this case), and photos taken on newer cell phones or digital cameras, many conditions can be diagnosed and managed appropriately.

Molluscum contagiosum is caused by the Molluscipox genus poxvirus and Is commonly seen in preschool and school-aged children. It can be passed through direct contact with infected individuals or spread by fomites. (In this case, the child may have picked up the virus by sharing a towel with an infected individual.)

The flesh-colored lesions are umbilicated or popular, and occur in clusters on the trunk, face, and extremities. Typically, the lesions will resolve spontaneously, but it may take several weeks to many months for resolution.

Given this lengthy time for spontaneous resolution, the risk of spreading to family members or other contacts, and the skin’s appearance, many patients choose to treat the lesions. Treatment options include curettage, cryosurgery, and laser. Available topical destructive agents include podophyllotoxin, trichloroacetic acid, benzoyl peroxide, potassium hydroxide, and cantharidin (which is from the blister beetle and often difficult to obtain). There also are naturopathic topical products and immune system modulators, including topical imiquimod. These treatments are commonly used, but are off-label for the treatment of molluscum contagiosum.

The family was counseled that there is debate about the effectiveness of imiquimod for molluscum contagiosum, but that some studies find it to be useful. In this case, the mother chose a prescription for imiquimod cream 5%, to be applied 3 times weekly at bedtime until the lesions resolved. (The cream can be used for up to 16 weeks.) The family was advised that erythema and irritation are expected adverse effects at the application site.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

While the eroded scabbed area in a raised lesion with pearly borders raised the suspicion of basal cell carcinoma (BCC), a superficial shave biopsy revealed that this was sclerosing basosquamous carcinoma.

Basosquamous carcinoma is an uncommon form of BCC. It features nests/clusters of basaloid cells arising from the basal layer of the epidermis (a hallmark of BCC), as well as keratinization, which is seen in squamous cell carcinoma (SCC). Basosquamous carcinoma occurs in the same areas as more common types of nonmelanoma skin cancers (NMSCs)—that is, sun-exposed areas. Not surprisingly, then, the highest risk areas for basosquamous carcinoma are the face, hands, arms, scalp (in those without hair), and back. People with occupational or intentional sun exposure through tanning have even higher rates of NMSC than the average population; patients using tanning booths exposing their entire bodies warrant more extensive physical exams.

Basosquamous carcinoma is one of the subtypes of BCC that is associated with a higher risk of local invasion, recurrence, and metastasis. Treatment with Mohs micrographic surgery (MMS) reduces the risk of recurrence to 4% (from 12% to 51%). Even with MMS, basosquamous carcinoma can be particularly challenging. It may require additional excision stages, as well as a larger than anticipated excision size at the time of surgery.

In this case, the patient was referred for MMS due to the high-risk nature of this lesion.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

While the eroded scabbed area in a raised lesion with pearly borders raised the suspicion of basal cell carcinoma (BCC), a superficial shave biopsy revealed that this was sclerosing basosquamous carcinoma.

Basosquamous carcinoma is an uncommon form of BCC. It features nests/clusters of basaloid cells arising from the basal layer of the epidermis (a hallmark of BCC), as well as keratinization, which is seen in squamous cell carcinoma (SCC). Basosquamous carcinoma occurs in the same areas as more common types of nonmelanoma skin cancers (NMSCs)—that is, sun-exposed areas. Not surprisingly, then, the highest risk areas for basosquamous carcinoma are the face, hands, arms, scalp (in those without hair), and back. People with occupational or intentional sun exposure through tanning have even higher rates of NMSC than the average population; patients using tanning booths exposing their entire bodies warrant more extensive physical exams.

Basosquamous carcinoma is one of the subtypes of BCC that is associated with a higher risk of local invasion, recurrence, and metastasis. Treatment with Mohs micrographic surgery (MMS) reduces the risk of recurrence to 4% (from 12% to 51%). Even with MMS, basosquamous carcinoma can be particularly challenging. It may require additional excision stages, as well as a larger than anticipated excision size at the time of surgery.

In this case, the patient was referred for MMS due to the high-risk nature of this lesion.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

While the eroded scabbed area in a raised lesion with pearly borders raised the suspicion of basal cell carcinoma (BCC), a superficial shave biopsy revealed that this was sclerosing basosquamous carcinoma.

Basosquamous carcinoma is an uncommon form of BCC. It features nests/clusters of basaloid cells arising from the basal layer of the epidermis (a hallmark of BCC), as well as keratinization, which is seen in squamous cell carcinoma (SCC). Basosquamous carcinoma occurs in the same areas as more common types of nonmelanoma skin cancers (NMSCs)—that is, sun-exposed areas. Not surprisingly, then, the highest risk areas for basosquamous carcinoma are the face, hands, arms, scalp (in those without hair), and back. People with occupational or intentional sun exposure through tanning have even higher rates of NMSC than the average population; patients using tanning booths exposing their entire bodies warrant more extensive physical exams.

Basosquamous carcinoma is one of the subtypes of BCC that is associated with a higher risk of local invasion, recurrence, and metastasis. Treatment with Mohs micrographic surgery (MMS) reduces the risk of recurrence to 4% (from 12% to 51%). Even with MMS, basosquamous carcinoma can be particularly challenging. It may require additional excision stages, as well as a larger than anticipated excision size at the time of surgery.

In this case, the patient was referred for MMS due to the high-risk nature of this lesion.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

This pink raised nodule underlying a scaly surface was suspicious for squamous cell carcinoma (SCC). Since this was a virtual visit, and the lesion required pathology due to the likelihood of cancer, the patient was brought into the clinic for additional evaluation. A broad-based deep shave biopsy was performed to remove the visible lesion. Pathology showed SCC in situ, with borders uninvolved.

Patients who have had AKs are extremely likely to develop additional AKs. A notable percentage of AKs will, over time, develop into SCC in situ and then invasive SCC if not treated. While cryosurgery of an SK should not result in SCC, it’s most likely that in this case, an AK adjacent to the SK progressed to the SCC in situ.

There are multiple treatments available for SCC in situ. Topical imiquimod has been shown to be somewhat effective in stimulating the immune system, thus leading to resolution of SCC in situ. But there is a significant risk of recurrence. Topical 5-FU can be utilized on a daily or twice daily basis for 2 weeks (or up to several months). The risk of recurrence ranges from 7% to 33%. Electrodesiccation and curettage is often used for SCC in situ, with recurrence rates of 2% to 19%. Cryosurgery for SCC in situ requires an aggressive freeze, with freeze times of up to 30 seconds. Photodynamic therapy also is an option; however, it requires multiple sessions and is more costly than other treatment options.

This patient’s borders were uninvolved on pathology, but it was possible that there was some residual SCC in situ due to the standard “bread loaf slicing” used for routine pathology. To treat possible residual SCC in situ at the wound site and surrounding tissue, the patient was given a prescription for topical 5-FU to apply twice daily for 6 weeks. The patient was instructed to return for follow-up in 6 months, or sooner, if any problems arose.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

This pink raised nodule underlying a scaly surface was suspicious for squamous cell carcinoma (SCC). Since this was a virtual visit, and the lesion required pathology due to the likelihood of cancer, the patient was brought into the clinic for additional evaluation. A broad-based deep shave biopsy was performed to remove the visible lesion. Pathology showed SCC in situ, with borders uninvolved.

Patients who have had AKs are extremely likely to develop additional AKs. A notable percentage of AKs will, over time, develop into SCC in situ and then invasive SCC if not treated. While cryosurgery of an SK should not result in SCC, it’s most likely that in this case, an AK adjacent to the SK progressed to the SCC in situ.

There are multiple treatments available for SCC in situ. Topical imiquimod has been shown to be somewhat effective in stimulating the immune system, thus leading to resolution of SCC in situ. But there is a significant risk of recurrence. Topical 5-FU can be utilized on a daily or twice daily basis for 2 weeks (or up to several months). The risk of recurrence ranges from 7% to 33%. Electrodesiccation and curettage is often used for SCC in situ, with recurrence rates of 2% to 19%. Cryosurgery for SCC in situ requires an aggressive freeze, with freeze times of up to 30 seconds. Photodynamic therapy also is an option; however, it requires multiple sessions and is more costly than other treatment options.

This patient’s borders were uninvolved on pathology, but it was possible that there was some residual SCC in situ due to the standard “bread loaf slicing” used for routine pathology. To treat possible residual SCC in situ at the wound site and surrounding tissue, the patient was given a prescription for topical 5-FU to apply twice daily for 6 weeks. The patient was instructed to return for follow-up in 6 months, or sooner, if any problems arose.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

This pink raised nodule underlying a scaly surface was suspicious for squamous cell carcinoma (SCC). Since this was a virtual visit, and the lesion required pathology due to the likelihood of cancer, the patient was brought into the clinic for additional evaluation. A broad-based deep shave biopsy was performed to remove the visible lesion. Pathology showed SCC in situ, with borders uninvolved.

Patients who have had AKs are extremely likely to develop additional AKs. A notable percentage of AKs will, over time, develop into SCC in situ and then invasive SCC if not treated. While cryosurgery of an SK should not result in SCC, it’s most likely that in this case, an AK adjacent to the SK progressed to the SCC in situ.

There are multiple treatments available for SCC in situ. Topical imiquimod has been shown to be somewhat effective in stimulating the immune system, thus leading to resolution of SCC in situ. But there is a significant risk of recurrence. Topical 5-FU can be utilized on a daily or twice daily basis for 2 weeks (or up to several months). The risk of recurrence ranges from 7% to 33%. Electrodesiccation and curettage is often used for SCC in situ, with recurrence rates of 2% to 19%. Cryosurgery for SCC in situ requires an aggressive freeze, with freeze times of up to 30 seconds. Photodynamic therapy also is an option; however, it requires multiple sessions and is more costly than other treatment options.

This patient’s borders were uninvolved on pathology, but it was possible that there was some residual SCC in situ due to the standard “bread loaf slicing” used for routine pathology. To treat possible residual SCC in situ at the wound site and surrounding tissue, the patient was given a prescription for topical 5-FU to apply twice daily for 6 weeks. The patient was instructed to return for follow-up in 6 months, or sooner, if any problems arose.

‌

Photo and text courtesy of Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

The abrupt onset of painful, violaceous coalescing papules, pustules, cysts, and nodules exclusively involving the centrofacial area with an overlying red-cyanotic erythema are the hallmarks of pyoderma faciale.

Pyoderma faciale is a rare disease that affects females in the second and third decades of life; 50% of these patients have a history of acne. The etiology of the condition remains unclear. Hormonal imbalance, inflammatory bowel disease, liver disease, and thyroid disease have been associated with the disorder. Ribavirin and interferon therapies for the treatment of hepatitis C along with high levels of vitamins (B₆ and B₁₂) have been identified as triggers. Culture of purulent drainage typically is sterile or may reveal commensal organisms.

The differential diagnosis includes acne fulminans and acne conglobata. Acne fulminans is not restricted to the face, as is pyoderma faciale, and it involves constitutional symptoms. Acne conglobata is a chronic process that affects males and females. It also involves purulent sinus tracts.

Prompt treatment of pyoderma faciale is essential to prevent widespread eruption, minimize the distress associated with the disfiguring nature of the disorder, and ultimately reduce scarring. Standard therapy consists of oral steroids (prednisone 1 mg/kg/d) for 1 week followed by a slow taper in combination with oral isotretinoin at a low dosage (0.2–0.5 mg/kg). The systemic retinoid is continued until all inflammatory lesions are healed.

In this case, a culture swab taken from the patient’s left cheek did not reveal any unexpected pathogens. The patient was started on oral doxycycline 100 mg bid and prednisone 50 mg/d tapered to 10 mg/d. She was counseled about the risks and benefits of isotretinoin and registered in the iPLEDGE system in anticipation of starting oral isotretinoin at 20 mg/d after negative pregnancy tests, 2 forms of contraception, and the 1-month qualification period.

‌

Photo courtesy of Catherine N. Tchanque-Fossuo, MD, MS, and text courtesy of Catherine N. Tchanque-Fossuo, MD, MS, Department of Dermatology, and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

The abrupt onset of painful, violaceous coalescing papules, pustules, cysts, and nodules exclusively involving the centrofacial area with an overlying red-cyanotic erythema are the hallmarks of pyoderma faciale.

Pyoderma faciale is a rare disease that affects females in the second and third decades of life; 50% of these patients have a history of acne. The etiology of the condition remains unclear. Hormonal imbalance, inflammatory bowel disease, liver disease, and thyroid disease have been associated with the disorder. Ribavirin and interferon therapies for the treatment of hepatitis C along with high levels of vitamins (B₆ and B₁₂) have been identified as triggers. Culture of purulent drainage typically is sterile or may reveal commensal organisms.

The differential diagnosis includes acne fulminans and acne conglobata. Acne fulminans is not restricted to the face, as is pyoderma faciale, and it involves constitutional symptoms. Acne conglobata is a chronic process that affects males and females. It also involves purulent sinus tracts.

Prompt treatment of pyoderma faciale is essential to prevent widespread eruption, minimize the distress associated with the disfiguring nature of the disorder, and ultimately reduce scarring. Standard therapy consists of oral steroids (prednisone 1 mg/kg/d) for 1 week followed by a slow taper in combination with oral isotretinoin at a low dosage (0.2–0.5 mg/kg). The systemic retinoid is continued until all inflammatory lesions are healed.

In this case, a culture swab taken from the patient’s left cheek did not reveal any unexpected pathogens. The patient was started on oral doxycycline 100 mg bid and prednisone 50 mg/d tapered to 10 mg/d. She was counseled about the risks and benefits of isotretinoin and registered in the iPLEDGE system in anticipation of starting oral isotretinoin at 20 mg/d after negative pregnancy tests, 2 forms of contraception, and the 1-month qualification period.

‌

Photo courtesy of Catherine N. Tchanque-Fossuo, MD, MS, and text courtesy of Catherine N. Tchanque-Fossuo, MD, MS, Department of Dermatology, and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

The abrupt onset of painful, violaceous coalescing papules, pustules, cysts, and nodules exclusively involving the centrofacial area with an overlying red-cyanotic erythema are the hallmarks of pyoderma faciale.

Pyoderma faciale is a rare disease that affects females in the second and third decades of life; 50% of these patients have a history of acne. The etiology of the condition remains unclear. Hormonal imbalance, inflammatory bowel disease, liver disease, and thyroid disease have been associated with the disorder. Ribavirin and interferon therapies for the treatment of hepatitis C along with high levels of vitamins (B₆ and B₁₂) have been identified as triggers. Culture of purulent drainage typically is sterile or may reveal commensal organisms.

The differential diagnosis includes acne fulminans and acne conglobata. Acne fulminans is not restricted to the face, as is pyoderma faciale, and it involves constitutional symptoms. Acne conglobata is a chronic process that affects males and females. It also involves purulent sinus tracts.

Prompt treatment of pyoderma faciale is essential to prevent widespread eruption, minimize the distress associated with the disfiguring nature of the disorder, and ultimately reduce scarring. Standard therapy consists of oral steroids (prednisone 1 mg/kg/d) for 1 week followed by a slow taper in combination with oral isotretinoin at a low dosage (0.2–0.5 mg/kg). The systemic retinoid is continued until all inflammatory lesions are healed.

In this case, a culture swab taken from the patient’s left cheek did not reveal any unexpected pathogens. The patient was started on oral doxycycline 100 mg bid and prednisone 50 mg/d tapered to 10 mg/d. She was counseled about the risks and benefits of isotretinoin and registered in the iPLEDGE system in anticipation of starting oral isotretinoin at 20 mg/d after negative pregnancy tests, 2 forms of contraception, and the 1-month qualification period.

‌

Photo courtesy of Catherine N. Tchanque-Fossuo, MD, MS, and text courtesy of Catherine N. Tchanque-Fossuo, MD, MS, Department of Dermatology, and Daniel Stulberg, MD, FAAFP, Department of Family and Community Medicine, University of New Mexico School of Medicine, Albuquerque.

The Wood’s lamp revealed a coral-red fluorescence in the interdigital spaces, which led to a diagnosis of erythrasma.

The coral-red fluorescence seen under the Wood’s lamp is due to porphyrins produced by Corynebacterium minutissimum. The organism invades the stratum corneum where it proliferates and causes erythrasma. Erythrasma typically appears as delineated, dry, red-brown patches in intertriginous areas, such as the axilla, groin, interdigital spaces, intergluteal cleft, perianal skin, and inframammary area.

Erythrasma affects 4% of the population; risk factors include poor hygiene, hyperhidrosis, obesity, warm climate, diabetes, and an immunocompromised state. The differential diagnosis for a pruritic rash between the toes includes tinea pedis and contact dermatitis.

First-line management of erythrasma includes both nonpharmacologic and pharmacologic modalities. Good hygiene and, depending on the area affected, loose-fitting cotton undergarments can help treat and prevent erythrasma.

Topical 2% miconazole bid for 2 weeks has resulted in clearance rates as high as 88%. Its affordable price, over-the-counter availability, and lack of adverse effects make miconazole a reasonable choice. It is also a smart treatment choice when erythrasma is coexisting with tinea because it can treat both conditions.

Topical 1% clindamycin or 2% erythromycin solution or gel bid for 2 weeks also can be used to treat the condition. However, given that topical antibiotics are more expensive than single-dose oral treatment and are no better than the oral formulations of these antibiotics, clarithromycin 1 g taken once orally may be preferred. Our patient was treated with a single dose of clarithromycin 1 g. At follow-up, her erythrasma was clear.

‌

This case was adapted from: Vo T, Usatine RP. Persistent rash on feet. J Fam Pract. 2018;67:107-109

The Wood’s lamp revealed a coral-red fluorescence in the interdigital spaces, which led to a diagnosis of erythrasma.

The coral-red fluorescence seen under the Wood’s lamp is due to porphyrins produced by Corynebacterium minutissimum. The organism invades the stratum corneum where it proliferates and causes erythrasma. Erythrasma typically appears as delineated, dry, red-brown patches in intertriginous areas, such as the axilla, groin, interdigital spaces, intergluteal cleft, perianal skin, and inframammary area.

Erythrasma affects 4% of the population; risk factors include poor hygiene, hyperhidrosis, obesity, warm climate, diabetes, and an immunocompromised state. The differential diagnosis for a pruritic rash between the toes includes tinea pedis and contact dermatitis.

First-line management of erythrasma includes both nonpharmacologic and pharmacologic modalities. Good hygiene and, depending on the area affected, loose-fitting cotton undergarments can help treat and prevent erythrasma.

Topical 2% miconazole bid for 2 weeks has resulted in clearance rates as high as 88%. Its affordable price, over-the-counter availability, and lack of adverse effects make miconazole a reasonable choice. It is also a smart treatment choice when erythrasma is coexisting with tinea because it can treat both conditions.

Topical 1% clindamycin or 2% erythromycin solution or gel bid for 2 weeks also can be used to treat the condition. However, given that topical antibiotics are more expensive than single-dose oral treatment and are no better than the oral formulations of these antibiotics, clarithromycin 1 g taken once orally may be preferred. Our patient was treated with a single dose of clarithromycin 1 g. At follow-up, her erythrasma was clear.

‌

This case was adapted from: Vo T, Usatine RP. Persistent rash on feet. J Fam Pract. 2018;67:107-109

The Wood’s lamp revealed a coral-red fluorescence in the interdigital spaces, which led to a diagnosis of erythrasma.

The coral-red fluorescence seen under the Wood’s lamp is due to porphyrins produced by Corynebacterium minutissimum. The organism invades the stratum corneum where it proliferates and causes erythrasma. Erythrasma typically appears as delineated, dry, red-brown patches in intertriginous areas, such as the axilla, groin, interdigital spaces, intergluteal cleft, perianal skin, and inframammary area.

Erythrasma affects 4% of the population; risk factors include poor hygiene, hyperhidrosis, obesity, warm climate, diabetes, and an immunocompromised state. The differential diagnosis for a pruritic rash between the toes includes tinea pedis and contact dermatitis.

First-line management of erythrasma includes both nonpharmacologic and pharmacologic modalities. Good hygiene and, depending on the area affected, loose-fitting cotton undergarments can help treat and prevent erythrasma.

Topical 2% miconazole bid for 2 weeks has resulted in clearance rates as high as 88%. Its affordable price, over-the-counter availability, and lack of adverse effects make miconazole a reasonable choice. It is also a smart treatment choice when erythrasma is coexisting with tinea because it can treat both conditions.

Topical 1% clindamycin or 2% erythromycin solution or gel bid for 2 weeks also can be used to treat the condition. However, given that topical antibiotics are more expensive than single-dose oral treatment and are no better than the oral formulations of these antibiotics, clarithromycin 1 g taken once orally may be preferred. Our patient was treated with a single dose of clarithromycin 1 g. At follow-up, her erythrasma was clear.

‌

This case was adapted from: Vo T, Usatine RP. Persistent rash on feet. J Fam Pract. 2018;67:107-109

References

1. US Preventive Services Task Force. Unhealthy drug use: screening [final recommendation statement]. Published June 9, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/drug-use-illicit-screening. Accessed July 28, 2020.
2. US Preventive Services Task Force. Illicit drug use in children, adolescents, and young adults: primary care-based interventions [final recommendation statement]. Published May 26, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/drug-use-illicit-primary-care-interventions-for-children-and-adolescents. Accessed July 28, 2020.
3. US Preventive Services Task Force. Prevention and cessation of tobacco use in children and adolescents: primary care interventions [final recommendation statement]. Published April 28, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/tobacco-and-nicotine-use-prevention-in-children-and-adolescents-primary-care-interventions. Accessed July 28, 2020.
4. National Institute on Drug Abuse. NIDA Quick Screen v 1.0. www.drugabuse.gov/sites/default/files/pdf/nmassist.pdf. Accessed July 28, 2020.
5. US Preventive Services Task Force. Tobacco smoking cessation in adults, including pregnant women: behavioral and pharmacotherapy interventions [update in progress]. Published September 21, 2015. https://uspreventiveservicestaskforce.org/uspstf/recommendation/tobacco-use-in-adults-and-pregnant-women-counseling-and-interventions. Accessed July 28, 2020.

References

1. US Preventive Services Task Force. Unhealthy drug use: screening [final recommendation statement]. Published June 9, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/drug-use-illicit-screening. Accessed July 28, 2020.
2. US Preventive Services Task Force. Illicit drug use in children, adolescents, and young adults: primary care-based interventions [final recommendation statement]. Published May 26, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/drug-use-illicit-primary-care-interventions-for-children-and-adolescents. Accessed July 28, 2020.
3. US Preventive Services Task Force. Prevention and cessation of tobacco use in children and adolescents: primary care interventions [final recommendation statement]. Published April 28, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/tobacco-and-nicotine-use-prevention-in-children-and-adolescents-primary-care-interventions. Accessed July 28, 2020.
4. National Institute on Drug Abuse. NIDA Quick Screen v 1.0. www.drugabuse.gov/sites/default/files/pdf/nmassist.pdf. Accessed July 28, 2020.
5. US Preventive Services Task Force. Tobacco smoking cessation in adults, including pregnant women: behavioral and pharmacotherapy interventions [update in progress]. Published September 21, 2015. https://uspreventiveservicestaskforce.org/uspstf/recommendation/tobacco-use-in-adults-and-pregnant-women-counseling-and-interventions. Accessed July 28, 2020.

References

1. US Preventive Services Task Force. Unhealthy drug use: screening [final recommendation statement]. Published June 9, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/drug-use-illicit-screening. Accessed July 28, 2020.
2. US Preventive Services Task Force. Illicit drug use in children, adolescents, and young adults: primary care-based interventions [final recommendation statement]. Published May 26, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/drug-use-illicit-primary-care-interventions-for-children-and-adolescents. Accessed July 28, 2020.
3. US Preventive Services Task Force. Prevention and cessation of tobacco use in children and adolescents: primary care interventions [final recommendation statement]. Published April 28, 2020. https://uspreventiveservicestaskforce.org/uspstf/recommendation/tobacco-and-nicotine-use-prevention-in-children-and-adolescents-primary-care-interventions. Accessed July 28, 2020.
4. National Institute on Drug Abuse. NIDA Quick Screen v 1.0. www.drugabuse.gov/sites/default/files/pdf/nmassist.pdf. Accessed July 28, 2020.
5. US Preventive Services Task Force. Tobacco smoking cessation in adults, including pregnant women: behavioral and pharmacotherapy interventions [update in progress]. Published September 21, 2015. https://uspreventiveservicestaskforce.org/uspstf/recommendation/tobacco-use-in-adults-and-pregnant-women-counseling-and-interventions. Accessed July 28, 2020.

The patient’s history of a recurrent wheal was consistent with a diagnosis of mastocytoma, the most common and least concerning form of cutaneous mastocytosis. Mastocytomas commonly appear in infants as 1 to 3 firm 1- to 5-cm papules (or a plaque) caused by a histamine release from a group of mast cells with abnormal growth receptors. When flaring, the surface may have a prominent orange peel texture because of tethered adnexal structures. When uninflamed, the skin surface may be slightly raised and flesh-colored to pink.

When first noticed, mastocytomas are easily mistaken for insect bites or congenital nevi. However, mastocytomas don’t resolve completely, as would an insect bite, and they become recurrently inflamed (spontaneously or with trauma). Inflammation that can be elicited with pressure or scratching is called Darrier sign and is helpful in making the diagnosis and distinguishing these lesions from congenital nevi.

Dermoscopy of a mastocytoma lacks signs of a melanocytic nevi, which further adds to the clinical diagnosis. Blood tests and biopsy are unnecessary, but if a biopsy is performed, it is important to mention the possibility of mast cell disease to the lab so that appropriate immunostaining for mast cells can be carried out.

Mastocytomas that appear in infancy usually resolve spontaneously in early childhood or by puberty, at the latest. If there is any notable itching or discomfort, oral antihistamines are helpful, as are topical steroids and topical tacrolimus. In this case, the diagnosis was made clinically and the patient’s parents were content to observe the area.

‌

Photos and text for Photo Rounds Friday courtesy of Jonathan Karnes, MD (copyright retained). Dr. Karnes is the medical director of MDFMR Dermatology Services, Augusta, ME.

The patient’s history of a recurrent wheal was consistent with a diagnosis of mastocytoma, the most common and least concerning form of cutaneous mastocytosis. Mastocytomas commonly appear in infants as 1 to 3 firm 1- to 5-cm papules (or a plaque) caused by a histamine release from a group of mast cells with abnormal growth receptors. When flaring, the surface may have a prominent orange peel texture because of tethered adnexal structures. When uninflamed, the skin surface may be slightly raised and flesh-colored to pink.

When first noticed, mastocytomas are easily mistaken for insect bites or congenital nevi. However, mastocytomas don’t resolve completely, as would an insect bite, and they become recurrently inflamed (spontaneously or with trauma). Inflammation that can be elicited with pressure or scratching is called Darrier sign and is helpful in making the diagnosis and distinguishing these lesions from congenital nevi.

Dermoscopy of a mastocytoma lacks signs of a melanocytic nevi, which further adds to the clinical diagnosis. Blood tests and biopsy are unnecessary, but if a biopsy is performed, it is important to mention the possibility of mast cell disease to the lab so that appropriate immunostaining for mast cells can be carried out.

Mastocytomas that appear in infancy usually resolve spontaneously in early childhood or by puberty, at the latest. If there is any notable itching or discomfort, oral antihistamines are helpful, as are topical steroids and topical tacrolimus. In this case, the diagnosis was made clinically and the patient’s parents were content to observe the area.

‌

Photos and text for Photo Rounds Friday courtesy of Jonathan Karnes, MD (copyright retained). Dr. Karnes is the medical director of MDFMR Dermatology Services, Augusta, ME.

The patient’s history of a recurrent wheal was consistent with a diagnosis of mastocytoma, the most common and least concerning form of cutaneous mastocytosis. Mastocytomas commonly appear in infants as 1 to 3 firm 1- to 5-cm papules (or a plaque) caused by a histamine release from a group of mast cells with abnormal growth receptors. When flaring, the surface may have a prominent orange peel texture because of tethered adnexal structures. When uninflamed, the skin surface may be slightly raised and flesh-colored to pink.

When first noticed, mastocytomas are easily mistaken for insect bites or congenital nevi. However, mastocytomas don’t resolve completely, as would an insect bite, and they become recurrently inflamed (spontaneously or with trauma). Inflammation that can be elicited with pressure or scratching is called Darrier sign and is helpful in making the diagnosis and distinguishing these lesions from congenital nevi.

Dermoscopy of a mastocytoma lacks signs of a melanocytic nevi, which further adds to the clinical diagnosis. Blood tests and biopsy are unnecessary, but if a biopsy is performed, it is important to mention the possibility of mast cell disease to the lab so that appropriate immunostaining for mast cells can be carried out.

Mastocytomas that appear in infancy usually resolve spontaneously in early childhood or by puberty, at the latest. If there is any notable itching or discomfort, oral antihistamines are helpful, as are topical steroids and topical tacrolimus. In this case, the diagnosis was made clinically and the patient’s parents were content to observe the area.

‌

Photos and text for Photo Rounds Friday courtesy of Jonathan Karnes, MD (copyright retained). Dr. Karnes is the medical director of MDFMR Dermatology Services, Augusta, ME.

THE CASE

A 50-year-old man presented to the primary care office for evaluation of foot pain. The day before, his left fifth toe had become exquisitely tender. He distinctly remembered that when he awoke, there was no discoloration or pain, but the toe later became “purple.” He denied any trauma. His medical record was notable for an extensive smoking history and a family history of early cardiovascular disease.

The patient appeared well but in obvious distress, secondary to the pain. His vital signs were unremarkable. His head, neck, lung, and cardiac exams revealed no abnormalities. Physical examination revealed a left fifth toe that was dusky purple and warm to the touch. Pain disproportionate to examination was noted on the anterior aspect of the toe, with limited range of motion. The patient walked with a compensated gait. Pulses were palpable on the posterior tibial (PT) and dorsalis pedis (DP) regions.

DIAGNOSIS

Based on our exam findings, we suspected a vascular injury and recommended an emergency consult by Podiatry, for which he was scheduled the following morning. The podiatric evaluation confirmed concern for a vascular injury and prompted a request for an emergent evaluation by Vascular Surgery.

The patient was seen emergently on Day 4 for a vascular surgery evaluation. Examination at that time showed a nearly absent femoral pulse on the left side and diminished and monophasic DP and PT pulses. His left foot demonstrated nonblanchable purpura that was clinically consistent with cholesterol embolization syndrome (CES).

We calculated the patient’s ankle-brachial index, and computed tomography angiography (CTA) was performed. While results were pending, the patient was started on aspirin 81 mg, clopidogrel 75 mg, and atorvastatin 40 mg, for a suspected slowly progressing iliac artery stenosis with a resulting acute atheroembolic event.

The CTA report showed a high-grade stenosis at the bifurcation of the left iliac artery, extending into both external and internal arteries. Of note, mild atherosclerotic disease without significant occlusion and runoff to the foot was observed into the tibial arteries. The stenosis extended into the profonda femoris artery, as well.

DISCUSSION

Atherosclerotic plaques are commonly encountered in patients with atherosclerotic disease; however, there are 2 varieties of emboli that arise from these plaques and one is often overlooked.^1-4 The more common of these variants, thromboemboli, originates from an atherosclerotic plaque and can become lodged in a medium or large vessel as a single embolus.

Continue to: By contrast...

By contrast, atheroemboli (commonly known as cholesterol emboli or cholesterol crystal embolization) originate from atherosclerotic plaques in the aorta or another large artery,⁵ which are prone to embolize if the underlying plaque experiences stress. As the plaque erodes, cholesterol crystals break off and embolize distally. These smaller crystals flood into the circulation, allowing a shower of emboli over time to occlude the arterioles. As occlusion spreads through the arterioles, multiple organ systems are affected. (It was previously thought that procedure-associated cases were common, but a literature review has not borne this out.⁵)

The shower of emboli often triggers a systemic inflammatory response, causing nondescript abnormalities of laboratory inflammatory markers.^6,7 Interestingly, hypereosinophilia is noted in about 80% of patients with CES.⁸It is not uncommon for atheroemboli of the lower extremity to manifest, as it did in this case, as “blue toe syndrome.”

No disease-specific testing. A confounding factor in validating the diagnosis of CES is the lack of disease-specific testing. However, CES should be considered in a patient with acute kidney injury and hypereosinophilia. Making the diagnosis requires a high degree of clinical suspicion. Any organ can be affected, although the brain, kidneys, gastrointestinal tract, skin, and skeletal muscles of the lower extremities are most frequently involved.⁹ If left undiagnosed, the results can be devastating: slow and chronic injury to a variety of organ systems over time, which may not be recognized as a harbinger of an insidious underlying process causing end-organ damage.

Technically, definitive diagnosis can be made by biopsy of an affected organ. However, biopsy’s utility is limited due to potential for sampling error, accessibility (as noted, the location of the involved organ[s] may make biopsy nearly impossible without additional surgical risk⁹), and risk of poor healing to the biopsy site.¹⁰

Treatment is two-fold: supportive care for the affected end organ and prevention of subsequent embolic events. The latter entails aggressive risk factor reduction strategies, such as smoking cessation, statin therapy, blood pressure control, and blood sugar control. Warfarin is not recommended for treatment of CES due to the risk of further plaque rupture, hemorrhage, acute and chronic renal failure, and cholesterol microembolization to other organs.^11,12

Continue to: Our patient

Our patient. After testing confirmed the diagnosis, the patient underwent an angioplasty. A stent was placed in his left iliac artery. He was continued on antiplatelet and statin therapy and was again counseled regarding smoking cessation.

THE TAKEAWAY

When patients present with symptoms suggestive of a vascular origin, consider CES. Although it can affect a multitude of organs, acute kidney injury and hypereosinophilia are the most common signs. Immediate intervention is required to save the affected organ; strategizing to reduce the risk for further embolic events is also key.

Warfarin is not recommended for treatment of cholesterol embolization syndrome due to the risk of further plaque rupture, hemorrhage, and cholesterol microembolization to other organs.

Prompt recognition of vascular emergencies, including those that are harbingers of atherosclerotic disease, is essential. As clinicians, it is imperative that we use all resources to address significant population health burdens. If CES is more prevalent than commonly thought, consideration should be given to increasing education about early detection and treatment of this disorder, including the reinforcement of primary prevention and aggressive treatment of risk factors for atherosclerotic cardiovascular disease.

CORRESPONDENCE
Meagan Vermeulen, MD, FAAFP, Department of Family Medicine, Rowan University School of Osteopathic Medicine, 42 East Laurel Road, Suite 2100A, Stratford, NJ 08084; vermeulen@rowan.edu

THE CASE

A 50-year-old man presented to the primary care office for evaluation of foot pain. The day before, his left fifth toe had become exquisitely tender. He distinctly remembered that when he awoke, there was no discoloration or pain, but the toe later became “purple.” He denied any trauma. His medical record was notable for an extensive smoking history and a family history of early cardiovascular disease.

The patient appeared well but in obvious distress, secondary to the pain. His vital signs were unremarkable. His head, neck, lung, and cardiac exams revealed no abnormalities. Physical examination revealed a left fifth toe that was dusky purple and warm to the touch. Pain disproportionate to examination was noted on the anterior aspect of the toe, with limited range of motion. The patient walked with a compensated gait. Pulses were palpable on the posterior tibial (PT) and dorsalis pedis (DP) regions.

DIAGNOSIS

Based on our exam findings, we suspected a vascular injury and recommended an emergency consult by Podiatry, for which he was scheduled the following morning. The podiatric evaluation confirmed concern for a vascular injury and prompted a request for an emergent evaluation by Vascular Surgery.

The patient was seen emergently on Day 4 for a vascular surgery evaluation. Examination at that time showed a nearly absent femoral pulse on the left side and diminished and monophasic DP and PT pulses. His left foot demonstrated nonblanchable purpura that was clinically consistent with cholesterol embolization syndrome (CES).

We calculated the patient’s ankle-brachial index, and computed tomography angiography (CTA) was performed. While results were pending, the patient was started on aspirin 81 mg, clopidogrel 75 mg, and atorvastatin 40 mg, for a suspected slowly progressing iliac artery stenosis with a resulting acute atheroembolic event.

The CTA report showed a high-grade stenosis at the bifurcation of the left iliac artery, extending into both external and internal arteries. Of note, mild atherosclerotic disease without significant occlusion and runoff to the foot was observed into the tibial arteries. The stenosis extended into the profonda femoris artery, as well.

DISCUSSION

Atherosclerotic plaques are commonly encountered in patients with atherosclerotic disease; however, there are 2 varieties of emboli that arise from these plaques and one is often overlooked.^1-4 The more common of these variants, thromboemboli, originates from an atherosclerotic plaque and can become lodged in a medium or large vessel as a single embolus.

Continue to: By contrast...

By contrast, atheroemboli (commonly known as cholesterol emboli or cholesterol crystal embolization) originate from atherosclerotic plaques in the aorta or another large artery,⁵ which are prone to embolize if the underlying plaque experiences stress. As the plaque erodes, cholesterol crystals break off and embolize distally. These smaller crystals flood into the circulation, allowing a shower of emboli over time to occlude the arterioles. As occlusion spreads through the arterioles, multiple organ systems are affected. (It was previously thought that procedure-associated cases were common, but a literature review has not borne this out.⁵)

The shower of emboli often triggers a systemic inflammatory response, causing nondescript abnormalities of laboratory inflammatory markers.^6,7 Interestingly, hypereosinophilia is noted in about 80% of patients with CES.⁸It is not uncommon for atheroemboli of the lower extremity to manifest, as it did in this case, as “blue toe syndrome.”

No disease-specific testing. A confounding factor in validating the diagnosis of CES is the lack of disease-specific testing. However, CES should be considered in a patient with acute kidney injury and hypereosinophilia. Making the diagnosis requires a high degree of clinical suspicion. Any organ can be affected, although the brain, kidneys, gastrointestinal tract, skin, and skeletal muscles of the lower extremities are most frequently involved.⁹ If left undiagnosed, the results can be devastating: slow and chronic injury to a variety of organ systems over time, which may not be recognized as a harbinger of an insidious underlying process causing end-organ damage.

Technically, definitive diagnosis can be made by biopsy of an affected organ. However, biopsy’s utility is limited due to potential for sampling error, accessibility (as noted, the location of the involved organ[s] may make biopsy nearly impossible without additional surgical risk⁹), and risk of poor healing to the biopsy site.¹⁰

Treatment is two-fold: supportive care for the affected end organ and prevention of subsequent embolic events. The latter entails aggressive risk factor reduction strategies, such as smoking cessation, statin therapy, blood pressure control, and blood sugar control. Warfarin is not recommended for treatment of CES due to the risk of further plaque rupture, hemorrhage, acute and chronic renal failure, and cholesterol microembolization to other organs.^11,12

Continue to: Our patient

Our patient. After testing confirmed the diagnosis, the patient underwent an angioplasty. A stent was placed in his left iliac artery. He was continued on antiplatelet and statin therapy and was again counseled regarding smoking cessation.

THE TAKEAWAY

When patients present with symptoms suggestive of a vascular origin, consider CES. Although it can affect a multitude of organs, acute kidney injury and hypereosinophilia are the most common signs. Immediate intervention is required to save the affected organ; strategizing to reduce the risk for further embolic events is also key.

Warfarin is not recommended for treatment of cholesterol embolization syndrome due to the risk of further plaque rupture, hemorrhage, and cholesterol microembolization to other organs.

Prompt recognition of vascular emergencies, including those that are harbingers of atherosclerotic disease, is essential. As clinicians, it is imperative that we use all resources to address significant population health burdens. If CES is more prevalent than commonly thought, consideration should be given to increasing education about early detection and treatment of this disorder, including the reinforcement of primary prevention and aggressive treatment of risk factors for atherosclerotic cardiovascular disease.

CORRESPONDENCE
Meagan Vermeulen, MD, FAAFP, Department of Family Medicine, Rowan University School of Osteopathic Medicine, 42 East Laurel Road, Suite 2100A, Stratford, NJ 08084; vermeulen@rowan.edu

THE CASE

A 50-year-old man presented to the primary care office for evaluation of foot pain. The day before, his left fifth toe had become exquisitely tender. He distinctly remembered that when he awoke, there was no discoloration or pain, but the toe later became “purple.” He denied any trauma. His medical record was notable for an extensive smoking history and a family history of early cardiovascular disease.

The patient appeared well but in obvious distress, secondary to the pain. His vital signs were unremarkable. His head, neck, lung, and cardiac exams revealed no abnormalities. Physical examination revealed a left fifth toe that was dusky purple and warm to the touch. Pain disproportionate to examination was noted on the anterior aspect of the toe, with limited range of motion. The patient walked with a compensated gait. Pulses were palpable on the posterior tibial (PT) and dorsalis pedis (DP) regions.

DIAGNOSIS

Based on our exam findings, we suspected a vascular injury and recommended an emergency consult by Podiatry, for which he was scheduled the following morning. The podiatric evaluation confirmed concern for a vascular injury and prompted a request for an emergent evaluation by Vascular Surgery.

The patient was seen emergently on Day 4 for a vascular surgery evaluation. Examination at that time showed a nearly absent femoral pulse on the left side and diminished and monophasic DP and PT pulses. His left foot demonstrated nonblanchable purpura that was clinically consistent with cholesterol embolization syndrome (CES).

We calculated the patient’s ankle-brachial index, and computed tomography angiography (CTA) was performed. While results were pending, the patient was started on aspirin 81 mg, clopidogrel 75 mg, and atorvastatin 40 mg, for a suspected slowly progressing iliac artery stenosis with a resulting acute atheroembolic event.

The CTA report showed a high-grade stenosis at the bifurcation of the left iliac artery, extending into both external and internal arteries. Of note, mild atherosclerotic disease without significant occlusion and runoff to the foot was observed into the tibial arteries. The stenosis extended into the profonda femoris artery, as well.

DISCUSSION

Atherosclerotic plaques are commonly encountered in patients with atherosclerotic disease; however, there are 2 varieties of emboli that arise from these plaques and one is often overlooked.^1-4 The more common of these variants, thromboemboli, originates from an atherosclerotic plaque and can become lodged in a medium or large vessel as a single embolus.

Continue to: By contrast...

By contrast, atheroemboli (commonly known as cholesterol emboli or cholesterol crystal embolization) originate from atherosclerotic plaques in the aorta or another large artery,⁵ which are prone to embolize if the underlying plaque experiences stress. As the plaque erodes, cholesterol crystals break off and embolize distally. These smaller crystals flood into the circulation, allowing a shower of emboli over time to occlude the arterioles. As occlusion spreads through the arterioles, multiple organ systems are affected. (It was previously thought that procedure-associated cases were common, but a literature review has not borne this out.⁵)

The shower of emboli often triggers a systemic inflammatory response, causing nondescript abnormalities of laboratory inflammatory markers.^6,7 Interestingly, hypereosinophilia is noted in about 80% of patients with CES.⁸It is not uncommon for atheroemboli of the lower extremity to manifest, as it did in this case, as “blue toe syndrome.”

No disease-specific testing. A confounding factor in validating the diagnosis of CES is the lack of disease-specific testing. However, CES should be considered in a patient with acute kidney injury and hypereosinophilia. Making the diagnosis requires a high degree of clinical suspicion. Any organ can be affected, although the brain, kidneys, gastrointestinal tract, skin, and skeletal muscles of the lower extremities are most frequently involved.⁹ If left undiagnosed, the results can be devastating: slow and chronic injury to a variety of organ systems over time, which may not be recognized as a harbinger of an insidious underlying process causing end-organ damage.

Technically, definitive diagnosis can be made by biopsy of an affected organ. However, biopsy’s utility is limited due to potential for sampling error, accessibility (as noted, the location of the involved organ[s] may make biopsy nearly impossible without additional surgical risk⁹), and risk of poor healing to the biopsy site.¹⁰

Treatment is two-fold: supportive care for the affected end organ and prevention of subsequent embolic events. The latter entails aggressive risk factor reduction strategies, such as smoking cessation, statin therapy, blood pressure control, and blood sugar control. Warfarin is not recommended for treatment of CES due to the risk of further plaque rupture, hemorrhage, acute and chronic renal failure, and cholesterol microembolization to other organs.^11,12

Continue to: Our patient

Our patient. After testing confirmed the diagnosis, the patient underwent an angioplasty. A stent was placed in his left iliac artery. He was continued on antiplatelet and statin therapy and was again counseled regarding smoking cessation.

THE TAKEAWAY

When patients present with symptoms suggestive of a vascular origin, consider CES. Although it can affect a multitude of organs, acute kidney injury and hypereosinophilia are the most common signs. Immediate intervention is required to save the affected organ; strategizing to reduce the risk for further embolic events is also key.

Warfarin is not recommended for treatment of cholesterol embolization syndrome due to the risk of further plaque rupture, hemorrhage, and cholesterol microembolization to other organs.

Prompt recognition of vascular emergencies, including those that are harbingers of atherosclerotic disease, is essential. As clinicians, it is imperative that we use all resources to address significant population health burdens. If CES is more prevalent than commonly thought, consideration should be given to increasing education about early detection and treatment of this disorder, including the reinforcement of primary prevention and aggressive treatment of risk factors for atherosclerotic cardiovascular disease.

CORRESPONDENCE
Meagan Vermeulen, MD, FAAFP, Department of Family Medicine, Rowan University School of Osteopathic Medicine, 42 East Laurel Road, Suite 2100A, Stratford, NJ 08084; vermeulen@rowan.edu

A 90-year-old man was admitted from the Emergency Department (ED) to our inpatient service for difficulty urinating and hematuria. In the ED, a complete blood count (CBC) with differential and a urinalysis were performed. CBC showed a mild normocytic anemia, consistent with the patient’s known chronic kidney disease. The urinalysis revealed moderate blood, trace ketones, proteinuria, small leukocyte esterases, positive nitrites, and more than 182 red blood cells—findings suspicious for a urinary tract infection. Computed tomography of the abdomen and pelvis was notable for a soft-tissue mass in the bladder.

He had a history of coronary artery disease (treated with stent placement), atrial fibrillation, congestive heart failure, hypothyroidism, gastroesophageal reflux disease, gastrointestinal bleeding, chronic obstructive pulmonary disease, a 60-pack-per-year history of tobacco dependence, chronic kidney disease, prostate cancer, benign prostatic hypertrophy, peripheral vascular disease, and gout. Medications included digoxin, metoprolol, torsemide, aspirin, levothyroxine, fluticasone, albuterol, omeprazole, diclofenac, escitalopram, and minocycline.

About 5 years earlier, doctors had discovered a popliteal thrombosis that required emergent thrombectomy of the infragenicular popliteal artery, thromboembolectomy of the right posterior tibial artery, graft angioplasty of the right posterior tibial artery, and right anterior fasciotomy for compartment syndrome.

Ten months later, an abscess formed at the incision site. His physician irrigated the popliteal wound and prescribed intravenous (IV) vancomycin. However, the patient developed an allergy and IV daptomycin was initiated and followed by chronic antibiotic suppression with oral minocycline 100 mg bid for about 3.5 years. Skin discoloration appeared within a year of starting the minocycline.

During his hospitalization on our service, we noted black pigmentation of both legs (FIGURE). He had intact strength and sensation in his legs, 1+ pitting edema, no pain upon palpation, and 2+ distal pulses. The patient was well appearing and in no acute distress.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

The patient’s clinical presentation of chronic blue-black hyperpigmentation on the anterior shins of both legs after a prolonged antibiotic course led us to conclude that this was an adverse effect of minocycline. Commonly, doctors use minocycline to treat acne, rosacea, and rheumatoid arthritis. In this case, it was used to provide chronic antimicrobial suppression.

Not an uncommon reaction for a patient like ours. One small study conducted in an orthopedic patient population found that 54% of patients receiving long-term minocycline suppression developed hyperpigmentation after a mean follow-up of nearly 5 years.¹ The hyperpigmentation is solely cosmetic and without known clinical complications, but it can be distressing for patients.

There are 3 types of minocycline-­induced hyperpigmentation:

• Type I is a circumscribed blue-black pigmentation that manifests in skin that previously was inflamed or scarred, such as facial acne scars.² Histopathologic findings include black pigment granules in macrophages and throughout the dermis that stain with Perls Prussian blue iron.³
• Type II (which our patient had) is circumscribed blue-black pigmentation that appears in previously normal skin of the forearms or lower legs—especially the shins.³ On histopathology, black pigment granules are found in the dermis with macrophages that stain with Perls Prussian blue iron and Fontana-Masson.³
• Type III is a diffuse muddy brown hyperpigmentation in previously normal, sun-exposed skin.² Histopathologic findings include increased melanin in basal keratinocytes and dermal melanophages that stain with Fontana-Masson.³

Monitor patients taking minocycline for early signs of pigmentation.

Types II and III may be related to cumulative dosing, whereas type I can occur at any point during treatment.²

Differential includes pigmentation disorders

The differential diagnosis includes Addison disease, argyria, hemochromatosis, and polycythemia vera, which all can cause diffuse blue-gray patches.⁴ Brown-violet pigmentation on sun-exposed areas, redness, and itching are more typical of Riehl melanosis.⁴

Continue to: Diltiazem

Diltiazem can produce slate-gray to blue-gray reticulated hyperpigmentation.⁵ Other drugs that can induce slate-gray macules or patches include amiodarone, chlorpromazine, imipramine, and desipramine.⁵

Treatment is simple, resolution takes time

The treatment for this condition is cessation of minocycline use. Pigmentation fades slowly and may persist for years. There has been successful treatment of type I and III minocycline-induced hyperpigmentation with the alexandrite 755 nm Q-switched laser combined with fractional photothermolysis.^3,6 Unfortunately, insurance coverage is limited because these treatments are cosmetic in nature.

Given that hyperpigmentation is a known adverse effect of minocycline use, it’s important to counsel patients about the possibility prior to initiating treatment. It’s also important to monitor for signs of changing pigmentation to prevent psychological distress.

In this case, a biopsy was deemed unnecessary, as the antibiotic was the most likely cause of the pigmentation. The patient’s outpatient dermatologist recommended changing therapy if a medically appropriate alternative was available. Doxycycline would have been a reasonable alternative; however, the patient died shortly after his presentation to our hospital due to his multiple comorbidities.

CORRESPONDENCE
Bich-May Nguyen, MD, MPH, 14023 Southwest Freeway, Sugar Land, TX 77478; Bich-May.Nguyen@memorialhermann.org

A 90-year-old man was admitted from the Emergency Department (ED) to our inpatient service for difficulty urinating and hematuria. In the ED, a complete blood count (CBC) with differential and a urinalysis were performed. CBC showed a mild normocytic anemia, consistent with the patient’s known chronic kidney disease. The urinalysis revealed moderate blood, trace ketones, proteinuria, small leukocyte esterases, positive nitrites, and more than 182 red blood cells—findings suspicious for a urinary tract infection. Computed tomography of the abdomen and pelvis was notable for a soft-tissue mass in the bladder.

He had a history of coronary artery disease (treated with stent placement), atrial fibrillation, congestive heart failure, hypothyroidism, gastroesophageal reflux disease, gastrointestinal bleeding, chronic obstructive pulmonary disease, a 60-pack-per-year history of tobacco dependence, chronic kidney disease, prostate cancer, benign prostatic hypertrophy, peripheral vascular disease, and gout. Medications included digoxin, metoprolol, torsemide, aspirin, levothyroxine, fluticasone, albuterol, omeprazole, diclofenac, escitalopram, and minocycline.

About 5 years earlier, doctors had discovered a popliteal thrombosis that required emergent thrombectomy of the infragenicular popliteal artery, thromboembolectomy of the right posterior tibial artery, graft angioplasty of the right posterior tibial artery, and right anterior fasciotomy for compartment syndrome.

Ten months later, an abscess formed at the incision site. His physician irrigated the popliteal wound and prescribed intravenous (IV) vancomycin. However, the patient developed an allergy and IV daptomycin was initiated and followed by chronic antibiotic suppression with oral minocycline 100 mg bid for about 3.5 years. Skin discoloration appeared within a year of starting the minocycline.

During his hospitalization on our service, we noted black pigmentation of both legs (FIGURE). He had intact strength and sensation in his legs, 1+ pitting edema, no pain upon palpation, and 2+ distal pulses. The patient was well appearing and in no acute distress.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

The patient’s clinical presentation of chronic blue-black hyperpigmentation on the anterior shins of both legs after a prolonged antibiotic course led us to conclude that this was an adverse effect of minocycline. Commonly, doctors use minocycline to treat acne, rosacea, and rheumatoid arthritis. In this case, it was used to provide chronic antimicrobial suppression.

Not an uncommon reaction for a patient like ours. One small study conducted in an orthopedic patient population found that 54% of patients receiving long-term minocycline suppression developed hyperpigmentation after a mean follow-up of nearly 5 years.¹ The hyperpigmentation is solely cosmetic and without known clinical complications, but it can be distressing for patients.

There are 3 types of minocycline-­induced hyperpigmentation:

• Type I is a circumscribed blue-black pigmentation that manifests in skin that previously was inflamed or scarred, such as facial acne scars.² Histopathologic findings include black pigment granules in macrophages and throughout the dermis that stain with Perls Prussian blue iron.³
• Type II (which our patient had) is circumscribed blue-black pigmentation that appears in previously normal skin of the forearms or lower legs—especially the shins.³ On histopathology, black pigment granules are found in the dermis with macrophages that stain with Perls Prussian blue iron and Fontana-Masson.³
• Type III is a diffuse muddy brown hyperpigmentation in previously normal, sun-exposed skin.² Histopathologic findings include increased melanin in basal keratinocytes and dermal melanophages that stain with Fontana-Masson.³

Monitor patients taking minocycline for early signs of pigmentation.

Types II and III may be related to cumulative dosing, whereas type I can occur at any point during treatment.²

Differential includes pigmentation disorders

The differential diagnosis includes Addison disease, argyria, hemochromatosis, and polycythemia vera, which all can cause diffuse blue-gray patches.⁴ Brown-violet pigmentation on sun-exposed areas, redness, and itching are more typical of Riehl melanosis.⁴

Continue to: Diltiazem

Diltiazem can produce slate-gray to blue-gray reticulated hyperpigmentation.⁵ Other drugs that can induce slate-gray macules or patches include amiodarone, chlorpromazine, imipramine, and desipramine.⁵

Treatment is simple, resolution takes time

The treatment for this condition is cessation of minocycline use. Pigmentation fades slowly and may persist for years. There has been successful treatment of type I and III minocycline-induced hyperpigmentation with the alexandrite 755 nm Q-switched laser combined with fractional photothermolysis.^3,6 Unfortunately, insurance coverage is limited because these treatments are cosmetic in nature.

Given that hyperpigmentation is a known adverse effect of minocycline use, it’s important to counsel patients about the possibility prior to initiating treatment. It’s also important to monitor for signs of changing pigmentation to prevent psychological distress.

In this case, a biopsy was deemed unnecessary, as the antibiotic was the most likely cause of the pigmentation. The patient’s outpatient dermatologist recommended changing therapy if a medically appropriate alternative was available. Doxycycline would have been a reasonable alternative; however, the patient died shortly after his presentation to our hospital due to his multiple comorbidities.

CORRESPONDENCE
Bich-May Nguyen, MD, MPH, 14023 Southwest Freeway, Sugar Land, TX 77478; Bich-May.Nguyen@memorialhermann.org

A 90-year-old man was admitted from the Emergency Department (ED) to our inpatient service for difficulty urinating and hematuria. In the ED, a complete blood count (CBC) with differential and a urinalysis were performed. CBC showed a mild normocytic anemia, consistent with the patient’s known chronic kidney disease. The urinalysis revealed moderate blood, trace ketones, proteinuria, small leukocyte esterases, positive nitrites, and more than 182 red blood cells—findings suspicious for a urinary tract infection. Computed tomography of the abdomen and pelvis was notable for a soft-tissue mass in the bladder.

He had a history of coronary artery disease (treated with stent placement), atrial fibrillation, congestive heart failure, hypothyroidism, gastroesophageal reflux disease, gastrointestinal bleeding, chronic obstructive pulmonary disease, a 60-pack-per-year history of tobacco dependence, chronic kidney disease, prostate cancer, benign prostatic hypertrophy, peripheral vascular disease, and gout. Medications included digoxin, metoprolol, torsemide, aspirin, levothyroxine, fluticasone, albuterol, omeprazole, diclofenac, escitalopram, and minocycline.

About 5 years earlier, doctors had discovered a popliteal thrombosis that required emergent thrombectomy of the infragenicular popliteal artery, thromboembolectomy of the right posterior tibial artery, graft angioplasty of the right posterior tibial artery, and right anterior fasciotomy for compartment syndrome.

Ten months later, an abscess formed at the incision site. His physician irrigated the popliteal wound and prescribed intravenous (IV) vancomycin. However, the patient developed an allergy and IV daptomycin was initiated and followed by chronic antibiotic suppression with oral minocycline 100 mg bid for about 3.5 years. Skin discoloration appeared within a year of starting the minocycline.

During his hospitalization on our service, we noted black pigmentation of both legs (FIGURE). He had intact strength and sensation in his legs, 1+ pitting edema, no pain upon palpation, and 2+ distal pulses. The patient was well appearing and in no acute distress.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

The patient’s clinical presentation of chronic blue-black hyperpigmentation on the anterior shins of both legs after a prolonged antibiotic course led us to conclude that this was an adverse effect of minocycline. Commonly, doctors use minocycline to treat acne, rosacea, and rheumatoid arthritis. In this case, it was used to provide chronic antimicrobial suppression.

Not an uncommon reaction for a patient like ours. One small study conducted in an orthopedic patient population found that 54% of patients receiving long-term minocycline suppression developed hyperpigmentation after a mean follow-up of nearly 5 years.¹ The hyperpigmentation is solely cosmetic and without known clinical complications, but it can be distressing for patients.

There are 3 types of minocycline-­induced hyperpigmentation:

• Type I is a circumscribed blue-black pigmentation that manifests in skin that previously was inflamed or scarred, such as facial acne scars.² Histopathologic findings include black pigment granules in macrophages and throughout the dermis that stain with Perls Prussian blue iron.³
• Type II (which our patient had) is circumscribed blue-black pigmentation that appears in previously normal skin of the forearms or lower legs—especially the shins.³ On histopathology, black pigment granules are found in the dermis with macrophages that stain with Perls Prussian blue iron and Fontana-Masson.³
• Type III is a diffuse muddy brown hyperpigmentation in previously normal, sun-exposed skin.² Histopathologic findings include increased melanin in basal keratinocytes and dermal melanophages that stain with Fontana-Masson.³

Monitor patients taking minocycline for early signs of pigmentation.

Types II and III may be related to cumulative dosing, whereas type I can occur at any point during treatment.²

Differential includes pigmentation disorders

The differential diagnosis includes Addison disease, argyria, hemochromatosis, and polycythemia vera, which all can cause diffuse blue-gray patches.⁴ Brown-violet pigmentation on sun-exposed areas, redness, and itching are more typical of Riehl melanosis.⁴

Continue to: Diltiazem

Diltiazem can produce slate-gray to blue-gray reticulated hyperpigmentation.⁵ Other drugs that can induce slate-gray macules or patches include amiodarone, chlorpromazine, imipramine, and desipramine.⁵

Treatment is simple, resolution takes time

The treatment for this condition is cessation of minocycline use. Pigmentation fades slowly and may persist for years. There has been successful treatment of type I and III minocycline-induced hyperpigmentation with the alexandrite 755 nm Q-switched laser combined with fractional photothermolysis.^3,6 Unfortunately, insurance coverage is limited because these treatments are cosmetic in nature.

Given that hyperpigmentation is a known adverse effect of minocycline use, it’s important to counsel patients about the possibility prior to initiating treatment. It’s also important to monitor for signs of changing pigmentation to prevent psychological distress.

In this case, a biopsy was deemed unnecessary, as the antibiotic was the most likely cause of the pigmentation. The patient’s outpatient dermatologist recommended changing therapy if a medically appropriate alternative was available. Doxycycline would have been a reasonable alternative; however, the patient died shortly after his presentation to our hospital due to his multiple comorbidities.

CORRESPONDENCE
Bich-May Nguyen, MD, MPH, 14023 Southwest Freeway, Sugar Land, TX 77478; Bich-May.Nguyen@memorialhermann.org