Richard P. Usatine, MD

A 48-year-old Hispanic woman came to the clinic as a new patient—her chief complaint was a rash that appeared on her face 3 months before and had recently spread to her chest and hands (FIGURES 1-3). It itched occasionally and seemed to worsen after exposure to the sun.

She also said that for the last month she had been feeling very weak—she had difficulty rising from a seated position and walking up the stairs to her apartment. She also felt as if her arms were heavy, making it difficult for her to brush and dry her hair in the morning.

The patient was otherwise healthy with no known medical conditions, and she was not taking any medications. Her family history was noncontributory.

A musculoskeletal examination showed the following:

Upper extremities:

• 4/5 strength shoulder abduction, internal and external rotation
• 5/5 strength biceps, triceps, wrist extension/flexion, grip
• 2+ biceps and triceps deep tendon reflexes bilaterally

Lower extremities:

• 4/5 strength hip flexors, quadriceps, hamstrings
• 5/5 dorsiflexion/plantar flexion
• 2+ patellar and ankle deep tendon reflexes bilaterally.

FIGURE 1
Facial rash and swelling

FIGURE 2
Rash spreading to chest

FIGURE 3
Plaques on knuckles

What is your diagnosis?
What diagnostic tests would you order for confirmation?

Diagnosis: Dermatomyositis

Dermatomyositis is a systemic disease classified as a type of idiopathic inflammatory myopathy. Dermatomyositis is a rare disease but one that may present initially to the family physician. It affects people at any age but is more commonly seen among children or adults aged >40 years.

Dermatomyositis involves the skin as well as skeletal muscle. Its cause is unknown; however, among those aged >50 years, malignancy may be an underlying cause. Cancers most commonly associated with dermatomyositis include those of the breast, ovary, lung, and gastrointestinal tract.

Skin manifestations may precede, follow, or present simultaneously with muscle involvement. Patients often complain of having difficulty ascending stairs, rising from a seated position, and performing overhead activities such as combing their hair. Patients may or may not have muscle tenderness and atrophy. Patients can have cutaneous involvement for more than a year before developing muscle weakness.¹

The dermatologic signs of dermatomyositis to watch for:

• Periorbital heliotrope erythema, usually associated with edema (FIGURE 1).
• Gottron’s papules—smooth, purple to red papules located over the knuckles, on the sides of the fingers, and sometimes on the elbows and knees. For adults, it is not uncommon to have plaques over the knuckles as opposed to the classic Gottron’s papules (FIGURE 3). In juvenile-onset dermatomyositis, distinct papules are much more evident upon presentation (FIGURE 4). Note that systemic lupus erythematosus (SLE) can present with a rash on the dorsum of the hands, but the rash spares the skin over the metacarpophalangeal and interphalangeal joints and affects the skin between the joints (FIGURE 5).
• Violaceous papular dermatitis with scale—may occur in localized areas, such as elbows and knees, or be diffusely distributed, starting off as a patchy erythema that coalesces and becomes slightly raised with scale. It tends to be confined to sun-exposed areas and worsens after sun exposure (FIGURE 2).
• Periungual erythema and telangiectasia—“moth-eaten” cuticles, a characteristic seen in other connective tissue diseases (FIGURE 6).¹

FIGURE 4
Papules on knuckles

FIGURE 5
Sparing interphalangeal joints

FIGURE 6
Cuticles with erythema

Differential diagnosis

Seborrheic dermatitis—white or yellow, greasy scales on an erythematous base with distribution on scalp, nasolabial folds and chest.

Atopic dermatitis—chronic history of pruritic papules or plaques with scale localized to flexural areas or may be generalized; lichenification may be seen.

Contact dermatitis—papules and vesicles that correspond to contact with allergen.

Polymorphous light eruption—clusters of erythematous, pruritic papules or vesicles occurring most frequently on the neck, anterior chest, arms, and forearms following sun exposure; most common among women in their twenties.

Lichen planus—pruritic, purple, polygonal papules (4 Ps) that may involve hair, nails, and mucous membranes in addition to the skin; more common among women, with onset between 30 to 60 years of age; may last months to years.

Psoriasis—well-demarcated papules and plaques on an erythematous base with thick, silvery scale; characteristically found on elbows, knees, scalp, nails, and genitalia.

Steroid myopathy—A side effect of systemic steroids, usually seen 4 to 6 weeks after beginning of treatment.

Dermatomyositis-like reaction—onset of similar skin findings with initiation of the following medications and improvement with discontinuation: penicillamine, nonsteroidal anti-inflammatory drugs, and carbamazepine.

Overlap syndrome—The term “overlap” denotes that certain signs are seen in both dermatomyositis and other connective tissue diseases such as scleroderma, rheumatoid arthritis, and lupus erythematosus. Scleroderma and dermatomyositis are the most commonly associated conditions and have been termed sclerodermatomyositis or mixed connective disease. In mixed connective tissue disease, features of SLE, scleroderma, and polymyositis are evident such as malar rash, alopecia, Raynaud’s phenomenon, waxy-appearing skin, and proximal muscle weakness.^1,2

Diagnostic tests: Muscle enzymes, EMG, biopsy

The diagnosis of dermatomyositis is confirmed by 3 laboratory tests: elevated muscle enzyme levels, electromyography, and muscle biopsy. A punch biopsy is helpful in differentiating dermatomyositis from other papulosquamous diseases such as lichen planus and psoriasis, but be careful as the histology of dermatomyositis is indistinguishable from cutaneous lupus erythematosus.

During the acute active phase, the following serum muscle enzymes may be elevated: creatine kinase (CK), lactate dehydrogenase (LDH), alanine aminotransferase (ALT or SGPT), aspartate aminotransferase (AST or SGOT), and aldolase. CK is elevated among 65% of patients and is most specific for muscle disease.³ Only one of the aforementioned enzymes may be elevated, so it is necessary to measure them all.

Measuring antibodies such as antinuclear antibody (ANA), Jo-1, SSA (Ro), SSB (La) supports the diagnosis if positive but dermatomyositis cannot be diagnosed solely on positive titers. It is not necessary to obtain an electromyograph or muscle biopsy for a patient with the characteristic skin findings and evidence of elevated muscle enzymes, as the diagnosis of dermatomyositis can be made with confidence. For a patient in whom the presentation is not as straightforward, it may be useful to obtain the electromyograph and muscle biopsy.

Management: Corticosteroids, watch for malignancy

Oral corticosteroids are the treatment of choice (strength of recommendation [SOR]: B).^1,4 Prednisone 0.5 to 1.0 mg/kg body weight per day has been recommended until muscle enzyme levels trend toward normal limits, at which time you can taper the dose. Steroid myopathy is a potential side effect of this treatment regimen; it may occur 4 to 6 weeks after therapy starts.

Several steroid-sparing agents such as methotrexate and cyclosporine are being prescribed by clinicians for dermomyositis but with little published evidence to support effectiveness. Methotrexate is an option for those who do not respond to prednisone or are in need of a steroid-sparing agent secondary to side effects (SOR: C).^2,3 A suggested regimen starts with 7.5 to 10 mg/wk, then increases the dose to 2.5 mg/wk until reaching a total dose of 25 mg/wk. The dose of prednisone should be decreased as the methotrexate dose increases. Azathioprine is another option to consider along with methotrexate, but choosing one agent over another or a combination of 2 agents remains empirical. With any of the immunosuppressants or immunomodulatory agents, it is important to look at their side-effect profiles and monitor the patient accordingly.

After initiating treatment, look for evidence of malignancy among those patients older than 50 years so as not to miss an underlying cancer as the cause for their dermatomyositis. For women without any risk factors, a complete annual physical exam—including pelvic, breast, and rectal exam—is sufficient.² It is not necessary to order expensive radiological studies blindly searching for malignancy, especially more than 2 years after the diagnosis is made. The greatest risk of malignancy occurs during the first year after diagnosis with a six-fold increase.¹ The risk drops during the second year and a patient’s risk for malignancy is comparable to the normal population in the years following. A mammogram and colonoscopy might be indicated after considering the patient’s age and family history.

The patient’s treatment and outcome

The patient was started on 60 mg of oral prednisone, taken in a single daily dose. She also began physical therapy twice a week in order to prevent muscle atrophy and maximize function. She took the prednisone for 1 month, at which time her creatine kinase level was trending towards normal. We then began slowly tapering the prednisone over the next 6 months.

She reported improvement in her strength 3 months after starting the systemic steroids. Little improvement was seen in the patient’s skin while on systemic steroids, but after prescribing 0.1% triamcinolone ointment, recommending a broad-spectrum sunscreen, and limiting sun exposure, the patient reported less erythema and edema.

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 48-year-old Hispanic woman came to the clinic as a new patient—her chief complaint was a rash that appeared on her face 3 months before and had recently spread to her chest and hands (FIGURES 1-3). It itched occasionally and seemed to worsen after exposure to the sun.

She also said that for the last month she had been feeling very weak—she had difficulty rising from a seated position and walking up the stairs to her apartment. She also felt as if her arms were heavy, making it difficult for her to brush and dry her hair in the morning.

The patient was otherwise healthy with no known medical conditions, and she was not taking any medications. Her family history was noncontributory.

A musculoskeletal examination showed the following:

Upper extremities:

• 4/5 strength shoulder abduction, internal and external rotation
• 5/5 strength biceps, triceps, wrist extension/flexion, grip
• 2+ biceps and triceps deep tendon reflexes bilaterally

Lower extremities:

• 4/5 strength hip flexors, quadriceps, hamstrings
• 5/5 dorsiflexion/plantar flexion
• 2+ patellar and ankle deep tendon reflexes bilaterally.

FIGURE 1
Facial rash and swelling

FIGURE 2
Rash spreading to chest

FIGURE 3
Plaques on knuckles

What is your diagnosis?
What diagnostic tests would you order for confirmation?

Diagnosis: Dermatomyositis

Dermatomyositis is a systemic disease classified as a type of idiopathic inflammatory myopathy. Dermatomyositis is a rare disease but one that may present initially to the family physician. It affects people at any age but is more commonly seen among children or adults aged >40 years.

Dermatomyositis involves the skin as well as skeletal muscle. Its cause is unknown; however, among those aged >50 years, malignancy may be an underlying cause. Cancers most commonly associated with dermatomyositis include those of the breast, ovary, lung, and gastrointestinal tract.

Skin manifestations may precede, follow, or present simultaneously with muscle involvement. Patients often complain of having difficulty ascending stairs, rising from a seated position, and performing overhead activities such as combing their hair. Patients may or may not have muscle tenderness and atrophy. Patients can have cutaneous involvement for more than a year before developing muscle weakness.¹

The dermatologic signs of dermatomyositis to watch for:

• Periorbital heliotrope erythema, usually associated with edema (FIGURE 1).
• Gottron’s papules—smooth, purple to red papules located over the knuckles, on the sides of the fingers, and sometimes on the elbows and knees. For adults, it is not uncommon to have plaques over the knuckles as opposed to the classic Gottron’s papules (FIGURE 3). In juvenile-onset dermatomyositis, distinct papules are much more evident upon presentation (FIGURE 4). Note that systemic lupus erythematosus (SLE) can present with a rash on the dorsum of the hands, but the rash spares the skin over the metacarpophalangeal and interphalangeal joints and affects the skin between the joints (FIGURE 5).
• Violaceous papular dermatitis with scale—may occur in localized areas, such as elbows and knees, or be diffusely distributed, starting off as a patchy erythema that coalesces and becomes slightly raised with scale. It tends to be confined to sun-exposed areas and worsens after sun exposure (FIGURE 2).
• Periungual erythema and telangiectasia—“moth-eaten” cuticles, a characteristic seen in other connective tissue diseases (FIGURE 6).¹

FIGURE 4
Papules on knuckles

FIGURE 5
Sparing interphalangeal joints

FIGURE 6
Cuticles with erythema

Differential diagnosis

Seborrheic dermatitis—white or yellow, greasy scales on an erythematous base with distribution on scalp, nasolabial folds and chest.

Atopic dermatitis—chronic history of pruritic papules or plaques with scale localized to flexural areas or may be generalized; lichenification may be seen.

Contact dermatitis—papules and vesicles that correspond to contact with allergen.

Polymorphous light eruption—clusters of erythematous, pruritic papules or vesicles occurring most frequently on the neck, anterior chest, arms, and forearms following sun exposure; most common among women in their twenties.

Lichen planus—pruritic, purple, polygonal papules (4 Ps) that may involve hair, nails, and mucous membranes in addition to the skin; more common among women, with onset between 30 to 60 years of age; may last months to years.

Psoriasis—well-demarcated papules and plaques on an erythematous base with thick, silvery scale; characteristically found on elbows, knees, scalp, nails, and genitalia.

Steroid myopathy—A side effect of systemic steroids, usually seen 4 to 6 weeks after beginning of treatment.

Dermatomyositis-like reaction—onset of similar skin findings with initiation of the following medications and improvement with discontinuation: penicillamine, nonsteroidal anti-inflammatory drugs, and carbamazepine.

Overlap syndrome—The term “overlap” denotes that certain signs are seen in both dermatomyositis and other connective tissue diseases such as scleroderma, rheumatoid arthritis, and lupus erythematosus. Scleroderma and dermatomyositis are the most commonly associated conditions and have been termed sclerodermatomyositis or mixed connective disease. In mixed connective tissue disease, features of SLE, scleroderma, and polymyositis are evident such as malar rash, alopecia, Raynaud’s phenomenon, waxy-appearing skin, and proximal muscle weakness.^1,2

Diagnostic tests: Muscle enzymes, EMG, biopsy

The diagnosis of dermatomyositis is confirmed by 3 laboratory tests: elevated muscle enzyme levels, electromyography, and muscle biopsy. A punch biopsy is helpful in differentiating dermatomyositis from other papulosquamous diseases such as lichen planus and psoriasis, but be careful as the histology of dermatomyositis is indistinguishable from cutaneous lupus erythematosus.

During the acute active phase, the following serum muscle enzymes may be elevated: creatine kinase (CK), lactate dehydrogenase (LDH), alanine aminotransferase (ALT or SGPT), aspartate aminotransferase (AST or SGOT), and aldolase. CK is elevated among 65% of patients and is most specific for muscle disease.³ Only one of the aforementioned enzymes may be elevated, so it is necessary to measure them all.

Measuring antibodies such as antinuclear antibody (ANA), Jo-1, SSA (Ro), SSB (La) supports the diagnosis if positive but dermatomyositis cannot be diagnosed solely on positive titers. It is not necessary to obtain an electromyograph or muscle biopsy for a patient with the characteristic skin findings and evidence of elevated muscle enzymes, as the diagnosis of dermatomyositis can be made with confidence. For a patient in whom the presentation is not as straightforward, it may be useful to obtain the electromyograph and muscle biopsy.

Management: Corticosteroids, watch for malignancy

Oral corticosteroids are the treatment of choice (strength of recommendation [SOR]: B).^1,4 Prednisone 0.5 to 1.0 mg/kg body weight per day has been recommended until muscle enzyme levels trend toward normal limits, at which time you can taper the dose. Steroid myopathy is a potential side effect of this treatment regimen; it may occur 4 to 6 weeks after therapy starts.

Several steroid-sparing agents such as methotrexate and cyclosporine are being prescribed by clinicians for dermomyositis but with little published evidence to support effectiveness. Methotrexate is an option for those who do not respond to prednisone or are in need of a steroid-sparing agent secondary to side effects (SOR: C).^2,3 A suggested regimen starts with 7.5 to 10 mg/wk, then increases the dose to 2.5 mg/wk until reaching a total dose of 25 mg/wk. The dose of prednisone should be decreased as the methotrexate dose increases. Azathioprine is another option to consider along with methotrexate, but choosing one agent over another or a combination of 2 agents remains empirical. With any of the immunosuppressants or immunomodulatory agents, it is important to look at their side-effect profiles and monitor the patient accordingly.

After initiating treatment, look for evidence of malignancy among those patients older than 50 years so as not to miss an underlying cancer as the cause for their dermatomyositis. For women without any risk factors, a complete annual physical exam—including pelvic, breast, and rectal exam—is sufficient.² It is not necessary to order expensive radiological studies blindly searching for malignancy, especially more than 2 years after the diagnosis is made. The greatest risk of malignancy occurs during the first year after diagnosis with a six-fold increase.¹ The risk drops during the second year and a patient’s risk for malignancy is comparable to the normal population in the years following. A mammogram and colonoscopy might be indicated after considering the patient’s age and family history.

The patient’s treatment and outcome

The patient was started on 60 mg of oral prednisone, taken in a single daily dose. She also began physical therapy twice a week in order to prevent muscle atrophy and maximize function. She took the prednisone for 1 month, at which time her creatine kinase level was trending towards normal. We then began slowly tapering the prednisone over the next 6 months.

She reported improvement in her strength 3 months after starting the systemic steroids. Little improvement was seen in the patient’s skin while on systemic steroids, but after prescribing 0.1% triamcinolone ointment, recommending a broad-spectrum sunscreen, and limiting sun exposure, the patient reported less erythema and edema.

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 48-year-old Hispanic woman came to the clinic as a new patient—her chief complaint was a rash that appeared on her face 3 months before and had recently spread to her chest and hands (FIGURES 1-3). It itched occasionally and seemed to worsen after exposure to the sun.

She also said that for the last month she had been feeling very weak—she had difficulty rising from a seated position and walking up the stairs to her apartment. She also felt as if her arms were heavy, making it difficult for her to brush and dry her hair in the morning.

The patient was otherwise healthy with no known medical conditions, and she was not taking any medications. Her family history was noncontributory.

A musculoskeletal examination showed the following:

Upper extremities:

• 4/5 strength shoulder abduction, internal and external rotation
• 5/5 strength biceps, triceps, wrist extension/flexion, grip
• 2+ biceps and triceps deep tendon reflexes bilaterally

Lower extremities:

• 4/5 strength hip flexors, quadriceps, hamstrings
• 5/5 dorsiflexion/plantar flexion
• 2+ patellar and ankle deep tendon reflexes bilaterally.

FIGURE 1
Facial rash and swelling

FIGURE 2
Rash spreading to chest

FIGURE 3
Plaques on knuckles

What is your diagnosis?
What diagnostic tests would you order for confirmation?

Diagnosis: Dermatomyositis

Dermatomyositis is a systemic disease classified as a type of idiopathic inflammatory myopathy. Dermatomyositis is a rare disease but one that may present initially to the family physician. It affects people at any age but is more commonly seen among children or adults aged >40 years.

Dermatomyositis involves the skin as well as skeletal muscle. Its cause is unknown; however, among those aged >50 years, malignancy may be an underlying cause. Cancers most commonly associated with dermatomyositis include those of the breast, ovary, lung, and gastrointestinal tract.

Skin manifestations may precede, follow, or present simultaneously with muscle involvement. Patients often complain of having difficulty ascending stairs, rising from a seated position, and performing overhead activities such as combing their hair. Patients may or may not have muscle tenderness and atrophy. Patients can have cutaneous involvement for more than a year before developing muscle weakness.¹

The dermatologic signs of dermatomyositis to watch for:

• Periorbital heliotrope erythema, usually associated with edema (FIGURE 1).
• Gottron’s papules—smooth, purple to red papules located over the knuckles, on the sides of the fingers, and sometimes on the elbows and knees. For adults, it is not uncommon to have plaques over the knuckles as opposed to the classic Gottron’s papules (FIGURE 3). In juvenile-onset dermatomyositis, distinct papules are much more evident upon presentation (FIGURE 4). Note that systemic lupus erythematosus (SLE) can present with a rash on the dorsum of the hands, but the rash spares the skin over the metacarpophalangeal and interphalangeal joints and affects the skin between the joints (FIGURE 5).
• Violaceous papular dermatitis with scale—may occur in localized areas, such as elbows and knees, or be diffusely distributed, starting off as a patchy erythema that coalesces and becomes slightly raised with scale. It tends to be confined to sun-exposed areas and worsens after sun exposure (FIGURE 2).
• Periungual erythema and telangiectasia—“moth-eaten” cuticles, a characteristic seen in other connective tissue diseases (FIGURE 6).¹

FIGURE 4
Papules on knuckles

FIGURE 5
Sparing interphalangeal joints

FIGURE 6
Cuticles with erythema

Differential diagnosis

Seborrheic dermatitis—white or yellow, greasy scales on an erythematous base with distribution on scalp, nasolabial folds and chest.

Atopic dermatitis—chronic history of pruritic papules or plaques with scale localized to flexural areas or may be generalized; lichenification may be seen.

Contact dermatitis—papules and vesicles that correspond to contact with allergen.

Polymorphous light eruption—clusters of erythematous, pruritic papules or vesicles occurring most frequently on the neck, anterior chest, arms, and forearms following sun exposure; most common among women in their twenties.

Lichen planus—pruritic, purple, polygonal papules (4 Ps) that may involve hair, nails, and mucous membranes in addition to the skin; more common among women, with onset between 30 to 60 years of age; may last months to years.

Psoriasis—well-demarcated papules and plaques on an erythematous base with thick, silvery scale; characteristically found on elbows, knees, scalp, nails, and genitalia.

Steroid myopathy—A side effect of systemic steroids, usually seen 4 to 6 weeks after beginning of treatment.

Dermatomyositis-like reaction—onset of similar skin findings with initiation of the following medications and improvement with discontinuation: penicillamine, nonsteroidal anti-inflammatory drugs, and carbamazepine.

Overlap syndrome—The term “overlap” denotes that certain signs are seen in both dermatomyositis and other connective tissue diseases such as scleroderma, rheumatoid arthritis, and lupus erythematosus. Scleroderma and dermatomyositis are the most commonly associated conditions and have been termed sclerodermatomyositis or mixed connective disease. In mixed connective tissue disease, features of SLE, scleroderma, and polymyositis are evident such as malar rash, alopecia, Raynaud’s phenomenon, waxy-appearing skin, and proximal muscle weakness.^1,2

Diagnostic tests: Muscle enzymes, EMG, biopsy

The diagnosis of dermatomyositis is confirmed by 3 laboratory tests: elevated muscle enzyme levels, electromyography, and muscle biopsy. A punch biopsy is helpful in differentiating dermatomyositis from other papulosquamous diseases such as lichen planus and psoriasis, but be careful as the histology of dermatomyositis is indistinguishable from cutaneous lupus erythematosus.

During the acute active phase, the following serum muscle enzymes may be elevated: creatine kinase (CK), lactate dehydrogenase (LDH), alanine aminotransferase (ALT or SGPT), aspartate aminotransferase (AST or SGOT), and aldolase. CK is elevated among 65% of patients and is most specific for muscle disease.³ Only one of the aforementioned enzymes may be elevated, so it is necessary to measure them all.

Measuring antibodies such as antinuclear antibody (ANA), Jo-1, SSA (Ro), SSB (La) supports the diagnosis if positive but dermatomyositis cannot be diagnosed solely on positive titers. It is not necessary to obtain an electromyograph or muscle biopsy for a patient with the characteristic skin findings and evidence of elevated muscle enzymes, as the diagnosis of dermatomyositis can be made with confidence. For a patient in whom the presentation is not as straightforward, it may be useful to obtain the electromyograph and muscle biopsy.

Management: Corticosteroids, watch for malignancy

Oral corticosteroids are the treatment of choice (strength of recommendation [SOR]: B).^1,4 Prednisone 0.5 to 1.0 mg/kg body weight per day has been recommended until muscle enzyme levels trend toward normal limits, at which time you can taper the dose. Steroid myopathy is a potential side effect of this treatment regimen; it may occur 4 to 6 weeks after therapy starts.

Several steroid-sparing agents such as methotrexate and cyclosporine are being prescribed by clinicians for dermomyositis but with little published evidence to support effectiveness. Methotrexate is an option for those who do not respond to prednisone or are in need of a steroid-sparing agent secondary to side effects (SOR: C).^2,3 A suggested regimen starts with 7.5 to 10 mg/wk, then increases the dose to 2.5 mg/wk until reaching a total dose of 25 mg/wk. The dose of prednisone should be decreased as the methotrexate dose increases. Azathioprine is another option to consider along with methotrexate, but choosing one agent over another or a combination of 2 agents remains empirical. With any of the immunosuppressants or immunomodulatory agents, it is important to look at their side-effect profiles and monitor the patient accordingly.

After initiating treatment, look for evidence of malignancy among those patients older than 50 years so as not to miss an underlying cancer as the cause for their dermatomyositis. For women without any risk factors, a complete annual physical exam—including pelvic, breast, and rectal exam—is sufficient.² It is not necessary to order expensive radiological studies blindly searching for malignancy, especially more than 2 years after the diagnosis is made. The greatest risk of malignancy occurs during the first year after diagnosis with a six-fold increase.¹ The risk drops during the second year and a patient’s risk for malignancy is comparable to the normal population in the years following. A mammogram and colonoscopy might be indicated after considering the patient’s age and family history.

The patient’s treatment and outcome

The patient was started on 60 mg of oral prednisone, taken in a single daily dose. She also began physical therapy twice a week in order to prevent muscle atrophy and maximize function. She took the prednisone for 1 month, at which time her creatine kinase level was trending towards normal. We then began slowly tapering the prednisone over the next 6 months.

She reported improvement in her strength 3 months after starting the systemic steroids. Little improvement was seen in the patient’s skin while on systemic steroids, but after prescribing 0.1% triamcinolone ointment, recommending a broad-spectrum sunscreen, and limiting sun exposure, the patient reported less erythema and edema.

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 56-year-old Hispanic woman came to the office distraught about the red rash on her face. The rash has been on her cheeks and nose for 5 years, but over the past 2 weeks she has developed red, mildly pruritic, and very tender papules that she calls granitos (the Spanish term for pimples). Foods do not make the rash better or worse, but it gets redder with sun exposure. She had not tried any lotions or sought medical attention until now, when she noticed that the papules were increasing in number but not size.

A few years ago she first noticed small blood vessels becoming more prominent, especially on her cheeks. The lesions have not bled or ulcerated. Her health has been generally good within the past year, with no recent infections. Review of systems indicated no changes in vision, upper respiratory illness, or systemic conditions. She is being treated for type 2 diabetes and hypertension. She does not have a family history of the same rash. FIGURES 1 AND 2 show erythema and telangiectasias distributed symmetrically on both cheeks and nose. A cluster of smooth papules were seen under the nose. No scales were noted.

FIGURE 1
Facial rash

FIGURE 2
Close-up

What is the diagnosis?
How would you treat this condition?

Diagnosis: Rosacea

The patient has rosacea. Rosacea is a common inflammatory skin condition characterized by erythema, edema, papules, pustules, or telangiectasias, most notably found on the concave portions of the cheeks, forehead, chin, and nose.¹ Its peak incidence is between the ages of 30 and 50 years.

Because of the marked flushing due to inflammation and capillary hyperreactivity and dilation, the rash has brought a certain degree of social embarrassment for some patients. Moreover, some equate rosacea with excessive alcohol consumption, but this is simply not true.

Although rosacea is a chronic disorder, it does alternate between periods of flare-up and remission. Flares may be triggered by stress, sun exposure, heat, hot drinks and spicy foods, alcohol, exercise, wind, and hot baths. In 50% of all rosacea patients, there are some ocular symptoms, ranging from mild dryness and grittiness to blepharitis, conjunctivitis, and even keratitis.²

Differential diagnosis

Although the differential diagnosis of rosacea is wide—acne, folliculitis, sarcoid, seborrheic dermatitis, and systemic lupus erythematosus [SLE]—there are unique characteristics that can help distinguish these from rosacea.

For example, the age of onset for rosacea tends to be 30 to 50 years, much later than the onset for acne vulgaris. Similarly, in acne vulgaris comedones are often present, but they are absent in rosacea.

Seborrheic dermatitis tends to produce scales while rosacea does not. Moreover, while both seborrheic dermatitis and rosacea can affect the central facial area, papules and telangiectasias are absent in the former while present with the latter.

SLE can be scarring, does not produce papules or pustules, and it spares the nasolabial folds and nose. Whereas rosacea has a more central face distribution, folliculitis typically presents with pustules visibly surrounding hair follicles and has associated tenderness to palpation and sometimes severe pruritus.

Causes and pathophysiology of rosacea

Although the cause of rosacea is unknown, its mechanism is understood to be nonspecific inflammation followed by dilation around follicles and hyperreactive capillaries. Oftentimes these dilated capillaries present as telangiectasias, which collectively exacerbate the red flushing. As the disorder progresses, diffuse hypertrophy of the connective tissue and sebaceous glands ensues.

Rosacea is more common in women than men (FIGURE 3). Men are typically more prone to the extreme forms of hyperplasia, which causes rhinophymatous rosacea (FIGURE 4)—eg,W C Fields’s nose.

Alcohol may accentuate erythema, but does not cause the disease. Sun exposure may precipitate an acute rosacea flare, but flare-ups can happen without sun exposure.

A significant increase in the hair follicle mite Demadex folliculorum is sometimes found in rosacea. It is theorized that these mites play a role because they incite an inflammatory or allergic reaction by mechanical blockage of follicles.

FIGURE 3
Papulopustular rosacea

FIGURE 4
Rhinophymatous rosacea

Stages of rosacea

There are 4 stages or subtypes of rosacea.

• Subtype 1: Erythematotelangiectatic rosacea. This stage is characterized by frequent mild to severe blushing with trace persistent central facial erythema in an individual.
• Subtype 2: Papulopustular rosacea (seen in FIGURES 1 , 2, AND 3). This is a highly vascular stage that involves longer periods of flushing than the first stage—often lasting from days to weeks. Minute telangiectasias and papules start to form by this stage, and some patients begin having very mild ocular complaints such as ocular grittiness or conjunctivitis.

Subtype 3: Phymatous rosacea. The third stage is also called the rhinophymatous stage. It is characterized by deepening shades of erythema and more papules and pustules. In the chronic forms of rosacea, hyperplasia of the sebaceous glands occurs, which forms a thickened confluent plaque of erythema at the tip of the nose known as rhinophyma. This hyperplasia can cause significant disfigurement to the forehead, eyelids, chin, and nose. The nasal disfiguration is seen more commonly in men than women (FIGURE 4).

Subtype 4: Ocular rosacea.The final or fourth stage is an advanced variation of rosacea that is characterized by impressive, severe flushing with persistent telangiectasias, papules, and pustules. At this point, more severe forms of conjunctivitis and blepharitis are more fullblown. The patient may complain of watery eyes, a foreign body sensation, burning, dryness, vision changes, and lid or periocular erythema.³

Diagnosis and treatment

Diagnostic tests

The diagnosis of rosacea is a clinical one. There is no confirmatory laboratory test. Biopsy is warranted only to rule out alternative diagnoses, since histopathological findings are not diagnostic. Scrapings may reveal Demadex folliculorum infection.¹

Treatment: Target the inflammation It is important to reassure patients about the benign nature of the disorder as well as explain that its cause is unknown. It may be useful to direct patients to information, such as web sites like those of the National Rosacea Society (www.rosacea.org). Advise patients to keep a daily diary to identify precipitating factors. These can include hot and humid weather, alcohol, hot beverages, spicy foods, and large hot meals. Suggest daily application of sunscreen, which protects against UVA and UVB rays.

Depending on the severity of the skin rosacea, the first-line treatment is an oral antibiotic (tetracycline or erythromycin) and/or topical metronidazole (0.75%–1.0%) twice daily. These antibiotics target the inflammation because rosacea is not a true infection. There is concern in the medical literature about how long-term use of antibiotics can promote drug resistance. Wolf et al⁴ proposed that once a patient’s lesions have improved after treatment with full-dose antibiotics, the clinician can consider switching to a lower dose and adding a topical agent such as metronidazole for maintenance.⁴ Studies have shown that 1.0% and 0.75% cream are equally effective.

TABLERosacea treatments

Another useful therapy is topical azelaic acid. While the evidence for scabicides in rosacea is limited, some clinicians use these medications for rosacea that is refractory to antibiotics. This is based on the idea that the Demadex mite is a causative agent in rosacea. If a patient has severe papulopustular disease refractory to antibiotics and topical treatments, the physician can start an oral isotretinoin regimen at a low dose of 0.1 to 0.5 mg/kg. Pulse-dye laser and electrosurgery can be used to treat the telangiectasias associated with rosacea.

A systematic review by Van Zuuren et al⁵ examined the efficacy of metronidazole, tetracycline, and azelaic acid in treating rosacea. Twenty-nine randomized controlled trials were found. Pooled data from 2 of the trials involving 174 participants indicated that, according to the participants, topical metronidazole was more effective than placebo (odds ratio [OR]=5.96; 95% confidence interval [CI], 2.95–12.06).

There was a definite improvement in the azelaic acid group; the rates of treatment success were approximately 70 to 80% versus 50% to 55% (OR=2.45; 95% CI, 1.82–3.28). Data pooled from 3 studies of oral tetracycline vs placebo involving 152 participants showed that, according to physicians, tetracycline was more effective than placebo (OR=6.06; 95% CI, 2.96–12.42).

Maintenance therapy

Because relapse occurs within weeks in about 25% of patients after the cessation of systemic therapy, topical therapy is usually used in an effort to maintain remission.⁶ The required duration of maintenance therapy is unknown, but a period of 6 months is generally advised. After this time, some patients report that they can keep their skin free of papulopustular lesions with topical therapy applied on alternate days or twice weekly, whereas others require repeated courses of systemic medication. After a few years, the disease may disappear spontaneously.

Patient management

Our patient was counseled about her rosacea and given the web site address for the National Rosacea Organization. She was advised to avoid sun exposure as much as possible and to use sunscreen and a hat to protect her face. She was prescribed tetracycline 500 mg and topical metronidazole to be used twice daily. Follow-up was set for 1 month. At that time, patient will be offered the option of electrocoagulation of her most prominent telangiectasias.

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 56-year-old Hispanic woman came to the office distraught about the red rash on her face. The rash has been on her cheeks and nose for 5 years, but over the past 2 weeks she has developed red, mildly pruritic, and very tender papules that she calls granitos (the Spanish term for pimples). Foods do not make the rash better or worse, but it gets redder with sun exposure. She had not tried any lotions or sought medical attention until now, when she noticed that the papules were increasing in number but not size.

A few years ago she first noticed small blood vessels becoming more prominent, especially on her cheeks. The lesions have not bled or ulcerated. Her health has been generally good within the past year, with no recent infections. Review of systems indicated no changes in vision, upper respiratory illness, or systemic conditions. She is being treated for type 2 diabetes and hypertension. She does not have a family history of the same rash. FIGURES 1 AND 2 show erythema and telangiectasias distributed symmetrically on both cheeks and nose. A cluster of smooth papules were seen under the nose. No scales were noted.

FIGURE 1
Facial rash

FIGURE 2
Close-up

What is the diagnosis?
How would you treat this condition?

Diagnosis: Rosacea

The patient has rosacea. Rosacea is a common inflammatory skin condition characterized by erythema, edema, papules, pustules, or telangiectasias, most notably found on the concave portions of the cheeks, forehead, chin, and nose.¹ Its peak incidence is between the ages of 30 and 50 years.

Because of the marked flushing due to inflammation and capillary hyperreactivity and dilation, the rash has brought a certain degree of social embarrassment for some patients. Moreover, some equate rosacea with excessive alcohol consumption, but this is simply not true.

Although rosacea is a chronic disorder, it does alternate between periods of flare-up and remission. Flares may be triggered by stress, sun exposure, heat, hot drinks and spicy foods, alcohol, exercise, wind, and hot baths. In 50% of all rosacea patients, there are some ocular symptoms, ranging from mild dryness and grittiness to blepharitis, conjunctivitis, and even keratitis.²

Differential diagnosis

Although the differential diagnosis of rosacea is wide—acne, folliculitis, sarcoid, seborrheic dermatitis, and systemic lupus erythematosus [SLE]—there are unique characteristics that can help distinguish these from rosacea.

For example, the age of onset for rosacea tends to be 30 to 50 years, much later than the onset for acne vulgaris. Similarly, in acne vulgaris comedones are often present, but they are absent in rosacea.

Seborrheic dermatitis tends to produce scales while rosacea does not. Moreover, while both seborrheic dermatitis and rosacea can affect the central facial area, papules and telangiectasias are absent in the former while present with the latter.

SLE can be scarring, does not produce papules or pustules, and it spares the nasolabial folds and nose. Whereas rosacea has a more central face distribution, folliculitis typically presents with pustules visibly surrounding hair follicles and has associated tenderness to palpation and sometimes severe pruritus.

Causes and pathophysiology of rosacea

Although the cause of rosacea is unknown, its mechanism is understood to be nonspecific inflammation followed by dilation around follicles and hyperreactive capillaries. Oftentimes these dilated capillaries present as telangiectasias, which collectively exacerbate the red flushing. As the disorder progresses, diffuse hypertrophy of the connective tissue and sebaceous glands ensues.

Rosacea is more common in women than men (FIGURE 3). Men are typically more prone to the extreme forms of hyperplasia, which causes rhinophymatous rosacea (FIGURE 4)—eg,W C Fields’s nose.

Alcohol may accentuate erythema, but does not cause the disease. Sun exposure may precipitate an acute rosacea flare, but flare-ups can happen without sun exposure.

A significant increase in the hair follicle mite Demadex folliculorum is sometimes found in rosacea. It is theorized that these mites play a role because they incite an inflammatory or allergic reaction by mechanical blockage of follicles.

FIGURE 3
Papulopustular rosacea

FIGURE 4
Rhinophymatous rosacea

Stages of rosacea

There are 4 stages or subtypes of rosacea.

• Subtype 1: Erythematotelangiectatic rosacea. This stage is characterized by frequent mild to severe blushing with trace persistent central facial erythema in an individual.
• Subtype 2: Papulopustular rosacea (seen in FIGURES 1 , 2, AND 3). This is a highly vascular stage that involves longer periods of flushing than the first stage—often lasting from days to weeks. Minute telangiectasias and papules start to form by this stage, and some patients begin having very mild ocular complaints such as ocular grittiness or conjunctivitis.

Subtype 3: Phymatous rosacea. The third stage is also called the rhinophymatous stage. It is characterized by deepening shades of erythema and more papules and pustules. In the chronic forms of rosacea, hyperplasia of the sebaceous glands occurs, which forms a thickened confluent plaque of erythema at the tip of the nose known as rhinophyma. This hyperplasia can cause significant disfigurement to the forehead, eyelids, chin, and nose. The nasal disfiguration is seen more commonly in men than women (FIGURE 4).

Subtype 4: Ocular rosacea.The final or fourth stage is an advanced variation of rosacea that is characterized by impressive, severe flushing with persistent telangiectasias, papules, and pustules. At this point, more severe forms of conjunctivitis and blepharitis are more fullblown. The patient may complain of watery eyes, a foreign body sensation, burning, dryness, vision changes, and lid or periocular erythema.³

Diagnosis and treatment

Diagnostic tests

The diagnosis of rosacea is a clinical one. There is no confirmatory laboratory test. Biopsy is warranted only to rule out alternative diagnoses, since histopathological findings are not diagnostic. Scrapings may reveal Demadex folliculorum infection.¹

Treatment: Target the inflammation It is important to reassure patients about the benign nature of the disorder as well as explain that its cause is unknown. It may be useful to direct patients to information, such as web sites like those of the National Rosacea Society (www.rosacea.org). Advise patients to keep a daily diary to identify precipitating factors. These can include hot and humid weather, alcohol, hot beverages, spicy foods, and large hot meals. Suggest daily application of sunscreen, which protects against UVA and UVB rays.

Depending on the severity of the skin rosacea, the first-line treatment is an oral antibiotic (tetracycline or erythromycin) and/or topical metronidazole (0.75%–1.0%) twice daily. These antibiotics target the inflammation because rosacea is not a true infection. There is concern in the medical literature about how long-term use of antibiotics can promote drug resistance. Wolf et al⁴ proposed that once a patient’s lesions have improved after treatment with full-dose antibiotics, the clinician can consider switching to a lower dose and adding a topical agent such as metronidazole for maintenance.⁴ Studies have shown that 1.0% and 0.75% cream are equally effective.

TABLERosacea treatments

Another useful therapy is topical azelaic acid. While the evidence for scabicides in rosacea is limited, some clinicians use these medications for rosacea that is refractory to antibiotics. This is based on the idea that the Demadex mite is a causative agent in rosacea. If a patient has severe papulopustular disease refractory to antibiotics and topical treatments, the physician can start an oral isotretinoin regimen at a low dose of 0.1 to 0.5 mg/kg. Pulse-dye laser and electrosurgery can be used to treat the telangiectasias associated with rosacea.

A systematic review by Van Zuuren et al⁵ examined the efficacy of metronidazole, tetracycline, and azelaic acid in treating rosacea. Twenty-nine randomized controlled trials were found. Pooled data from 2 of the trials involving 174 participants indicated that, according to the participants, topical metronidazole was more effective than placebo (odds ratio [OR]=5.96; 95% confidence interval [CI], 2.95–12.06).

There was a definite improvement in the azelaic acid group; the rates of treatment success were approximately 70 to 80% versus 50% to 55% (OR=2.45; 95% CI, 1.82–3.28). Data pooled from 3 studies of oral tetracycline vs placebo involving 152 participants showed that, according to physicians, tetracycline was more effective than placebo (OR=6.06; 95% CI, 2.96–12.42).

Maintenance therapy

Because relapse occurs within weeks in about 25% of patients after the cessation of systemic therapy, topical therapy is usually used in an effort to maintain remission.⁶ The required duration of maintenance therapy is unknown, but a period of 6 months is generally advised. After this time, some patients report that they can keep their skin free of papulopustular lesions with topical therapy applied on alternate days or twice weekly, whereas others require repeated courses of systemic medication. After a few years, the disease may disappear spontaneously.

Patient management

Our patient was counseled about her rosacea and given the web site address for the National Rosacea Organization. She was advised to avoid sun exposure as much as possible and to use sunscreen and a hat to protect her face. She was prescribed tetracycline 500 mg and topical metronidazole to be used twice daily. Follow-up was set for 1 month. At that time, patient will be offered the option of electrocoagulation of her most prominent telangiectasias.

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 56-year-old Hispanic woman came to the office distraught about the red rash on her face. The rash has been on her cheeks and nose for 5 years, but over the past 2 weeks she has developed red, mildly pruritic, and very tender papules that she calls granitos (the Spanish term for pimples). Foods do not make the rash better or worse, but it gets redder with sun exposure. She had not tried any lotions or sought medical attention until now, when she noticed that the papules were increasing in number but not size.

A few years ago she first noticed small blood vessels becoming more prominent, especially on her cheeks. The lesions have not bled or ulcerated. Her health has been generally good within the past year, with no recent infections. Review of systems indicated no changes in vision, upper respiratory illness, or systemic conditions. She is being treated for type 2 diabetes and hypertension. She does not have a family history of the same rash. FIGURES 1 AND 2 show erythema and telangiectasias distributed symmetrically on both cheeks and nose. A cluster of smooth papules were seen under the nose. No scales were noted.

FIGURE 1
Facial rash

FIGURE 2
Close-up

What is the diagnosis?
How would you treat this condition?

Diagnosis: Rosacea

The patient has rosacea. Rosacea is a common inflammatory skin condition characterized by erythema, edema, papules, pustules, or telangiectasias, most notably found on the concave portions of the cheeks, forehead, chin, and nose.¹ Its peak incidence is between the ages of 30 and 50 years.

Because of the marked flushing due to inflammation and capillary hyperreactivity and dilation, the rash has brought a certain degree of social embarrassment for some patients. Moreover, some equate rosacea with excessive alcohol consumption, but this is simply not true.

Although rosacea is a chronic disorder, it does alternate between periods of flare-up and remission. Flares may be triggered by stress, sun exposure, heat, hot drinks and spicy foods, alcohol, exercise, wind, and hot baths. In 50% of all rosacea patients, there are some ocular symptoms, ranging from mild dryness and grittiness to blepharitis, conjunctivitis, and even keratitis.²

Differential diagnosis

Although the differential diagnosis of rosacea is wide—acne, folliculitis, sarcoid, seborrheic dermatitis, and systemic lupus erythematosus [SLE]—there are unique characteristics that can help distinguish these from rosacea.

For example, the age of onset for rosacea tends to be 30 to 50 years, much later than the onset for acne vulgaris. Similarly, in acne vulgaris comedones are often present, but they are absent in rosacea.

Seborrheic dermatitis tends to produce scales while rosacea does not. Moreover, while both seborrheic dermatitis and rosacea can affect the central facial area, papules and telangiectasias are absent in the former while present with the latter.

SLE can be scarring, does not produce papules or pustules, and it spares the nasolabial folds and nose. Whereas rosacea has a more central face distribution, folliculitis typically presents with pustules visibly surrounding hair follicles and has associated tenderness to palpation and sometimes severe pruritus.

Causes and pathophysiology of rosacea

Although the cause of rosacea is unknown, its mechanism is understood to be nonspecific inflammation followed by dilation around follicles and hyperreactive capillaries. Oftentimes these dilated capillaries present as telangiectasias, which collectively exacerbate the red flushing. As the disorder progresses, diffuse hypertrophy of the connective tissue and sebaceous glands ensues.

Rosacea is more common in women than men (FIGURE 3). Men are typically more prone to the extreme forms of hyperplasia, which causes rhinophymatous rosacea (FIGURE 4)—eg,W C Fields’s nose.

Alcohol may accentuate erythema, but does not cause the disease. Sun exposure may precipitate an acute rosacea flare, but flare-ups can happen without sun exposure.

A significant increase in the hair follicle mite Demadex folliculorum is sometimes found in rosacea. It is theorized that these mites play a role because they incite an inflammatory or allergic reaction by mechanical blockage of follicles.

FIGURE 3
Papulopustular rosacea

FIGURE 4
Rhinophymatous rosacea

Stages of rosacea

There are 4 stages or subtypes of rosacea.

• Subtype 1: Erythematotelangiectatic rosacea. This stage is characterized by frequent mild to severe blushing with trace persistent central facial erythema in an individual.
• Subtype 2: Papulopustular rosacea (seen in FIGURES 1 , 2, AND 3). This is a highly vascular stage that involves longer periods of flushing than the first stage—often lasting from days to weeks. Minute telangiectasias and papules start to form by this stage, and some patients begin having very mild ocular complaints such as ocular grittiness or conjunctivitis.

Subtype 3: Phymatous rosacea. The third stage is also called the rhinophymatous stage. It is characterized by deepening shades of erythema and more papules and pustules. In the chronic forms of rosacea, hyperplasia of the sebaceous glands occurs, which forms a thickened confluent plaque of erythema at the tip of the nose known as rhinophyma. This hyperplasia can cause significant disfigurement to the forehead, eyelids, chin, and nose. The nasal disfiguration is seen more commonly in men than women (FIGURE 4).

Subtype 4: Ocular rosacea.The final or fourth stage is an advanced variation of rosacea that is characterized by impressive, severe flushing with persistent telangiectasias, papules, and pustules. At this point, more severe forms of conjunctivitis and blepharitis are more fullblown. The patient may complain of watery eyes, a foreign body sensation, burning, dryness, vision changes, and lid or periocular erythema.³

Diagnosis and treatment

Diagnostic tests

The diagnosis of rosacea is a clinical one. There is no confirmatory laboratory test. Biopsy is warranted only to rule out alternative diagnoses, since histopathological findings are not diagnostic. Scrapings may reveal Demadex folliculorum infection.¹

Treatment: Target the inflammation It is important to reassure patients about the benign nature of the disorder as well as explain that its cause is unknown. It may be useful to direct patients to information, such as web sites like those of the National Rosacea Society (www.rosacea.org). Advise patients to keep a daily diary to identify precipitating factors. These can include hot and humid weather, alcohol, hot beverages, spicy foods, and large hot meals. Suggest daily application of sunscreen, which protects against UVA and UVB rays.

Depending on the severity of the skin rosacea, the first-line treatment is an oral antibiotic (tetracycline or erythromycin) and/or topical metronidazole (0.75%–1.0%) twice daily. These antibiotics target the inflammation because rosacea is not a true infection. There is concern in the medical literature about how long-term use of antibiotics can promote drug resistance. Wolf et al⁴ proposed that once a patient’s lesions have improved after treatment with full-dose antibiotics, the clinician can consider switching to a lower dose and adding a topical agent such as metronidazole for maintenance.⁴ Studies have shown that 1.0% and 0.75% cream are equally effective.

TABLERosacea treatments

Another useful therapy is topical azelaic acid. While the evidence for scabicides in rosacea is limited, some clinicians use these medications for rosacea that is refractory to antibiotics. This is based on the idea that the Demadex mite is a causative agent in rosacea. If a patient has severe papulopustular disease refractory to antibiotics and topical treatments, the physician can start an oral isotretinoin regimen at a low dose of 0.1 to 0.5 mg/kg. Pulse-dye laser and electrosurgery can be used to treat the telangiectasias associated with rosacea.

A systematic review by Van Zuuren et al⁵ examined the efficacy of metronidazole, tetracycline, and azelaic acid in treating rosacea. Twenty-nine randomized controlled trials were found. Pooled data from 2 of the trials involving 174 participants indicated that, according to the participants, topical metronidazole was more effective than placebo (odds ratio [OR]=5.96; 95% confidence interval [CI], 2.95–12.06).

There was a definite improvement in the azelaic acid group; the rates of treatment success were approximately 70 to 80% versus 50% to 55% (OR=2.45; 95% CI, 1.82–3.28). Data pooled from 3 studies of oral tetracycline vs placebo involving 152 participants showed that, according to physicians, tetracycline was more effective than placebo (OR=6.06; 95% CI, 2.96–12.42).

Maintenance therapy

Because relapse occurs within weeks in about 25% of patients after the cessation of systemic therapy, topical therapy is usually used in an effort to maintain remission.⁶ The required duration of maintenance therapy is unknown, but a period of 6 months is generally advised. After this time, some patients report that they can keep their skin free of papulopustular lesions with topical therapy applied on alternate days or twice weekly, whereas others require repeated courses of systemic medication. After a few years, the disease may disappear spontaneously.

Patient management

Our patient was counseled about her rosacea and given the web site address for the National Rosacea Organization. She was advised to avoid sun exposure as much as possible and to use sunscreen and a hat to protect her face. She was prescribed tetracycline 500 mg and topical metronidazole to be used twice daily. Follow-up was set for 1 month. At that time, patient will be offered the option of electrocoagulation of her most prominent telangiectasias.

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 24-year-old woman, pregnant with a fetus at 22 weeks gestational age, came to the OB triage area with abdominal pain, nausea, and vomiting. She described a sharp pain that began the night before, starting at the umbilicus and radiating toward her right side; she rated it 7 out of 10.

The patient said there had been no contractions, vaginal bleeding or fluid leaking, or dysuria. She reported having GERD at times. She experienced chills the day before, but no fever. She had similar pain 1 month before that resolved spontaneously, and for which a cause was never determined. She had nothing significant in her medical history; family history was noncontributory.

On examination, she was afebrile, normotensive, and in no apparent distress. Her heart and lungs were normal. Her abdomen was soft and gravid with a fundal height of 22 cm. Bowel sounds were present in all 4 quadrants. Fetal heart tones were normal, and there was no indication of contractions. Her abdomen was diffusely tender, with significant tenderness to deep palpation in the right upper quadrant at first. There was no rebound or guarding. The psoas sign was negative. The obturator sign was positive, with increased pain 4 out of 10 in the right lower quadrant. There were no abdominal masses. Digital rectal examination revealed no rectal masses, and a guaiac stool test result was negative. A few hours later, the tenderness seemed to move toward the right lower quadrant (FIGURES 1 AND 2).

What is the most likely diagnosis?
How do the ultrasound images help you make the diagnosis?

FIGURE 1
Ultrasound of RLQ

FIGURE 2
A second ultrasound of RLQ

Differential diagnosis

The differential diagnosis of abdominal pain in a gravid patient includes placental abruption, cholecystitis, pancreatitis, appendicitis, intussusception, pyelonephritis, round ligament syndrome, hydronephrosis, ovarian torsion, uterine fibroid degeneration, ovarian cysts or tumors, intra-abdominal and rectus muscle abscesses, and Crohn’s disease with diffuse peritoneal inflammation. Given the location of the pain and the lack of vaginal bleeding, the most likely diagnoses are cholecystitis and appendicitis.

Making the diagnosis

We performed several laboratory analyses, including a complete blood count, chemistry panel (including electrolytes and liver function studies), amylase, lipase, and a urinalysis. The test results were all normal. She had a white blood cell count of 15,000/μL, which can be normal in pregnancy. The initial evaluating physician had obtained a right upper quadrant ultrasound, which showed no gallstones or bilateral hydronephrosis; unfortunately, no attempt was made to visualize the right lower quadrant or appendix at that time.

In light of the physical exam findings and the absence of gallstones, the patient was admitted to rule out appendicitis. The surgery team at the university hospital was consulted. They requested a computed tomography (CT) scan of the abdomen with and without contrast. To avoid the risk of radiation to the fetus, the family medicine team spoke with Radiology to obtain another ultrasound.

The ultrasound showed an enlarged and inflamed appendix with a transverse diameter of 13 mm (normal is <6 mm) (FIGURES 3 AND 4).¹ A graded compression technique was used to assess the appendix. This involves using pressure of the ultrasound probe starting above the area of tenderness and working toward the tender area while scanning for the appendix. This showed obvious peristalsis in the cecum and no movement within the appendix, indicating obstruction or inflammation.

FIGURE 3
Appendix: Longitudinal view

FIGURE 4
Appendix: Transverse view

Patient management and outcome

An open appendectomy was performed. The appendix was inflamed and enlarged as suspected. The histology showed neutrophilic infiltration of mucosa, muscle, and serosa (FIGURE 5). Postoperatively, the patient recovered in Labor and Delivery to monitor for possible preterm labor. She did not develop any signs or symptoms of preterm labor, and was transferred to a regular antepartum floor after being observed for 6 hours.

She did well during her hospitalization, and was sent home on post-op day 2. Her abdominal pain had resolved, and she had very little post-op tenderness.

FIGURE 5
Histology

Discussion: Appendicitis in pregnancy

Acute appendicitis is the most common condition requiring surgery during pregnancy.² Suspected appendicitis accounts for nearly two thirds of all nonobstetric exploratory laparotomies performed during pregnancy; most cases occur in the second and third trimesters.

The incidence of appendicitis is 0.4 to 1.4 per 1000 pregnancies.² Although the incidence of appendicitis in not increased during pregnancy, rupture of the appendix occurs 2 to 3 times more frequently in pregnancy secondary to delays in diagnosis and operation. Maternal and perinatal mortality and morbidity rates are greatly increased when appendicitis is complicated by peritonitis.

A difficult diagnosis

Diagnosis is difficult because many symptoms are considered to be normal during pregnancy. Many times, pain in the right lower quadrant of the abdomen may be attributed to round ligament pain or urinary tract infection. After the first trimester, the appendix is gradually displaced above McBurney’s point, with horizontal rotation of its base. This upward displacement occurs until the eighth month of gestation, when more than 90% of appendices lie above the iliac crest, and 80% rotate upward and toward the right subcostal area.^2,3

The most consistent clinical symptom encountered in pregnant women with appendicitis is vague right-sided abdominal pain.² Depending on the gestation, muscle guarding and rebound tenderness may or may not be present. Nausea, vomiting, and anorexia are usually present as in the nonpregnant patient. Twenty five percent of pregnant patients with appendicitis are afebrile, as our patient was.^2,4

The leukocytosis of pregnancy makes it difficult to determine if there is an infection. Not all pregnant patients with appendicitis will have a white blood cell count greater than 16,000/μL, but approximately 75% of them will have a left shift in the differential.² A urinalysis may reveal pyuria and hematuria and can mislead the physician to explain the symptoms as pyelonephritis.²

Treatment: Appendectomy, antibiotics if needed

Treatment of nonperforated acute appendicitis in pregnancy is appendectomy. In the first trimester, a laparoscopic appendectomy may be performed.² Intravenous antibiotics are indicated with perforation, peritonitis or abscess formation.^2,5

Tocolysis is unnecessary in uncomplicated appendicitis, but may be indicated if the patient goes into labor after surgery. In the late third trimester, with perforation or peritonitis, a cesarean section is indicated.

Evaluation is imperative

Fetal loss may occur in association with preterm labor and delivery or with generalized peritonitis and sepsis, and occurs only rarely in uncomplicated appendicitis. Fetal loss appears to be more closely associated with severity of appendicitis than with surgical intervention.^2,5,6

Imaging test characteristics: Is sonography enough?

Thus, it is imperative that any pregnant patient that comes in to the hospital or clinic with abdominal pain be evaluated for appendicitis. Ultrasound was a valuable diagnostic tool in this case and saved both the patient and developing fetus the radiation exposure of a CT scan. Ultrasound has a high specificity for diagnosing appendicitis if the appendix is visualized with abnormal findings. However, the sensitivity is not as high as CT, and failure to visualize the appendix adequately would have required a decision between appendectomy on clinical grounds only or going through with the CT scan.

The sensitivity, specificity, and positive and negative predictive values for ultrasonography and CT scans in the diagnosis of appendicitis are given in the TABLE (level of evidence [LOE]=1a).⁷

In a prospective study of patients with clinical signs and symptoms of acute appendicitis using a graded compression technique of ultrasonography, sonographic testing was as accurate as the focused unenhanced single-detector helical CT. The primary sonographic criterion for diagnosing acute appendicitis was an incompressible appendix with a transverse outer diameter of 6 mm or larger, as seen in this patient. The sensitivity of CT and sonography was 76% and 79%, respectively; the specificity was 83% and 78%; the accuracy was 78% and 78%; the positive predictive value was 90% and 87%; and the negative predictive value was 64% and 65% (LOE=2a).⁸

In conclusion, it is reasonable to use graded compression ultrasonography in a pregnant woman with suspected appendicitis. If the suspicion for appendicitis is high, a negative result may still need further evaluation with a CT or ultimately lead to abdominal surgery despite negative imaging studies.

TABLEUltrasound and CT in the diagnosis of appendicitis

Corresponding Author
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 24-year-old woman, pregnant with a fetus at 22 weeks gestational age, came to the OB triage area with abdominal pain, nausea, and vomiting. She described a sharp pain that began the night before, starting at the umbilicus and radiating toward her right side; she rated it 7 out of 10.

The patient said there had been no contractions, vaginal bleeding or fluid leaking, or dysuria. She reported having GERD at times. She experienced chills the day before, but no fever. She had similar pain 1 month before that resolved spontaneously, and for which a cause was never determined. She had nothing significant in her medical history; family history was noncontributory.

On examination, she was afebrile, normotensive, and in no apparent distress. Her heart and lungs were normal. Her abdomen was soft and gravid with a fundal height of 22 cm. Bowel sounds were present in all 4 quadrants. Fetal heart tones were normal, and there was no indication of contractions. Her abdomen was diffusely tender, with significant tenderness to deep palpation in the right upper quadrant at first. There was no rebound or guarding. The psoas sign was negative. The obturator sign was positive, with increased pain 4 out of 10 in the right lower quadrant. There were no abdominal masses. Digital rectal examination revealed no rectal masses, and a guaiac stool test result was negative. A few hours later, the tenderness seemed to move toward the right lower quadrant (FIGURES 1 AND 2).

What is the most likely diagnosis?
How do the ultrasound images help you make the diagnosis?

FIGURE 1
Ultrasound of RLQ

FIGURE 2
A second ultrasound of RLQ

Differential diagnosis

The differential diagnosis of abdominal pain in a gravid patient includes placental abruption, cholecystitis, pancreatitis, appendicitis, intussusception, pyelonephritis, round ligament syndrome, hydronephrosis, ovarian torsion, uterine fibroid degeneration, ovarian cysts or tumors, intra-abdominal and rectus muscle abscesses, and Crohn’s disease with diffuse peritoneal inflammation. Given the location of the pain and the lack of vaginal bleeding, the most likely diagnoses are cholecystitis and appendicitis.

Making the diagnosis

We performed several laboratory analyses, including a complete blood count, chemistry panel (including electrolytes and liver function studies), amylase, lipase, and a urinalysis. The test results were all normal. She had a white blood cell count of 15,000/μL, which can be normal in pregnancy. The initial evaluating physician had obtained a right upper quadrant ultrasound, which showed no gallstones or bilateral hydronephrosis; unfortunately, no attempt was made to visualize the right lower quadrant or appendix at that time.

In light of the physical exam findings and the absence of gallstones, the patient was admitted to rule out appendicitis. The surgery team at the university hospital was consulted. They requested a computed tomography (CT) scan of the abdomen with and without contrast. To avoid the risk of radiation to the fetus, the family medicine team spoke with Radiology to obtain another ultrasound.

The ultrasound showed an enlarged and inflamed appendix with a transverse diameter of 13 mm (normal is <6 mm) (FIGURES 3 AND 4).¹ A graded compression technique was used to assess the appendix. This involves using pressure of the ultrasound probe starting above the area of tenderness and working toward the tender area while scanning for the appendix. This showed obvious peristalsis in the cecum and no movement within the appendix, indicating obstruction or inflammation.

FIGURE 3
Appendix: Longitudinal view

FIGURE 4
Appendix: Transverse view

Patient management and outcome

An open appendectomy was performed. The appendix was inflamed and enlarged as suspected. The histology showed neutrophilic infiltration of mucosa, muscle, and serosa (FIGURE 5). Postoperatively, the patient recovered in Labor and Delivery to monitor for possible preterm labor. She did not develop any signs or symptoms of preterm labor, and was transferred to a regular antepartum floor after being observed for 6 hours.

She did well during her hospitalization, and was sent home on post-op day 2. Her abdominal pain had resolved, and she had very little post-op tenderness.

FIGURE 5
Histology

Discussion: Appendicitis in pregnancy

Acute appendicitis is the most common condition requiring surgery during pregnancy.² Suspected appendicitis accounts for nearly two thirds of all nonobstetric exploratory laparotomies performed during pregnancy; most cases occur in the second and third trimesters.

The incidence of appendicitis is 0.4 to 1.4 per 1000 pregnancies.² Although the incidence of appendicitis in not increased during pregnancy, rupture of the appendix occurs 2 to 3 times more frequently in pregnancy secondary to delays in diagnosis and operation. Maternal and perinatal mortality and morbidity rates are greatly increased when appendicitis is complicated by peritonitis.

A difficult diagnosis

Diagnosis is difficult because many symptoms are considered to be normal during pregnancy. Many times, pain in the right lower quadrant of the abdomen may be attributed to round ligament pain or urinary tract infection. After the first trimester, the appendix is gradually displaced above McBurney’s point, with horizontal rotation of its base. This upward displacement occurs until the eighth month of gestation, when more than 90% of appendices lie above the iliac crest, and 80% rotate upward and toward the right subcostal area.^2,3

The most consistent clinical symptom encountered in pregnant women with appendicitis is vague right-sided abdominal pain.² Depending on the gestation, muscle guarding and rebound tenderness may or may not be present. Nausea, vomiting, and anorexia are usually present as in the nonpregnant patient. Twenty five percent of pregnant patients with appendicitis are afebrile, as our patient was.^2,4

The leukocytosis of pregnancy makes it difficult to determine if there is an infection. Not all pregnant patients with appendicitis will have a white blood cell count greater than 16,000/μL, but approximately 75% of them will have a left shift in the differential.² A urinalysis may reveal pyuria and hematuria and can mislead the physician to explain the symptoms as pyelonephritis.²

Treatment: Appendectomy, antibiotics if needed

Treatment of nonperforated acute appendicitis in pregnancy is appendectomy. In the first trimester, a laparoscopic appendectomy may be performed.² Intravenous antibiotics are indicated with perforation, peritonitis or abscess formation.^2,5

Tocolysis is unnecessary in uncomplicated appendicitis, but may be indicated if the patient goes into labor after surgery. In the late third trimester, with perforation or peritonitis, a cesarean section is indicated.

Evaluation is imperative

Fetal loss may occur in association with preterm labor and delivery or with generalized peritonitis and sepsis, and occurs only rarely in uncomplicated appendicitis. Fetal loss appears to be more closely associated with severity of appendicitis than with surgical intervention.^2,5,6

Imaging test characteristics: Is sonography enough?

Thus, it is imperative that any pregnant patient that comes in to the hospital or clinic with abdominal pain be evaluated for appendicitis. Ultrasound was a valuable diagnostic tool in this case and saved both the patient and developing fetus the radiation exposure of a CT scan. Ultrasound has a high specificity for diagnosing appendicitis if the appendix is visualized with abnormal findings. However, the sensitivity is not as high as CT, and failure to visualize the appendix adequately would have required a decision between appendectomy on clinical grounds only or going through with the CT scan.

The sensitivity, specificity, and positive and negative predictive values for ultrasonography and CT scans in the diagnosis of appendicitis are given in the TABLE (level of evidence [LOE]=1a).⁷

In a prospective study of patients with clinical signs and symptoms of acute appendicitis using a graded compression technique of ultrasonography, sonographic testing was as accurate as the focused unenhanced single-detector helical CT. The primary sonographic criterion for diagnosing acute appendicitis was an incompressible appendix with a transverse outer diameter of 6 mm or larger, as seen in this patient. The sensitivity of CT and sonography was 76% and 79%, respectively; the specificity was 83% and 78%; the accuracy was 78% and 78%; the positive predictive value was 90% and 87%; and the negative predictive value was 64% and 65% (LOE=2a).⁸

In conclusion, it is reasonable to use graded compression ultrasonography in a pregnant woman with suspected appendicitis. If the suspicion for appendicitis is high, a negative result may still need further evaluation with a CT or ultimately lead to abdominal surgery despite negative imaging studies.

TABLEUltrasound and CT in the diagnosis of appendicitis

Corresponding Author
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 24-year-old woman, pregnant with a fetus at 22 weeks gestational age, came to the OB triage area with abdominal pain, nausea, and vomiting. She described a sharp pain that began the night before, starting at the umbilicus and radiating toward her right side; she rated it 7 out of 10.

The patient said there had been no contractions, vaginal bleeding or fluid leaking, or dysuria. She reported having GERD at times. She experienced chills the day before, but no fever. She had similar pain 1 month before that resolved spontaneously, and for which a cause was never determined. She had nothing significant in her medical history; family history was noncontributory.

On examination, she was afebrile, normotensive, and in no apparent distress. Her heart and lungs were normal. Her abdomen was soft and gravid with a fundal height of 22 cm. Bowel sounds were present in all 4 quadrants. Fetal heart tones were normal, and there was no indication of contractions. Her abdomen was diffusely tender, with significant tenderness to deep palpation in the right upper quadrant at first. There was no rebound or guarding. The psoas sign was negative. The obturator sign was positive, with increased pain 4 out of 10 in the right lower quadrant. There were no abdominal masses. Digital rectal examination revealed no rectal masses, and a guaiac stool test result was negative. A few hours later, the tenderness seemed to move toward the right lower quadrant (FIGURES 1 AND 2).

What is the most likely diagnosis?
How do the ultrasound images help you make the diagnosis?

FIGURE 1
Ultrasound of RLQ

FIGURE 2
A second ultrasound of RLQ

Differential diagnosis

The differential diagnosis of abdominal pain in a gravid patient includes placental abruption, cholecystitis, pancreatitis, appendicitis, intussusception, pyelonephritis, round ligament syndrome, hydronephrosis, ovarian torsion, uterine fibroid degeneration, ovarian cysts or tumors, intra-abdominal and rectus muscle abscesses, and Crohn’s disease with diffuse peritoneal inflammation. Given the location of the pain and the lack of vaginal bleeding, the most likely diagnoses are cholecystitis and appendicitis.

Making the diagnosis

We performed several laboratory analyses, including a complete blood count, chemistry panel (including electrolytes and liver function studies), amylase, lipase, and a urinalysis. The test results were all normal. She had a white blood cell count of 15,000/μL, which can be normal in pregnancy. The initial evaluating physician had obtained a right upper quadrant ultrasound, which showed no gallstones or bilateral hydronephrosis; unfortunately, no attempt was made to visualize the right lower quadrant or appendix at that time.

In light of the physical exam findings and the absence of gallstones, the patient was admitted to rule out appendicitis. The surgery team at the university hospital was consulted. They requested a computed tomography (CT) scan of the abdomen with and without contrast. To avoid the risk of radiation to the fetus, the family medicine team spoke with Radiology to obtain another ultrasound.

The ultrasound showed an enlarged and inflamed appendix with a transverse diameter of 13 mm (normal is <6 mm) (FIGURES 3 AND 4).¹ A graded compression technique was used to assess the appendix. This involves using pressure of the ultrasound probe starting above the area of tenderness and working toward the tender area while scanning for the appendix. This showed obvious peristalsis in the cecum and no movement within the appendix, indicating obstruction or inflammation.

FIGURE 3
Appendix: Longitudinal view

FIGURE 4
Appendix: Transverse view

Patient management and outcome

An open appendectomy was performed. The appendix was inflamed and enlarged as suspected. The histology showed neutrophilic infiltration of mucosa, muscle, and serosa (FIGURE 5). Postoperatively, the patient recovered in Labor and Delivery to monitor for possible preterm labor. She did not develop any signs or symptoms of preterm labor, and was transferred to a regular antepartum floor after being observed for 6 hours.

She did well during her hospitalization, and was sent home on post-op day 2. Her abdominal pain had resolved, and she had very little post-op tenderness.

FIGURE 5
Histology

Discussion: Appendicitis in pregnancy

Acute appendicitis is the most common condition requiring surgery during pregnancy.² Suspected appendicitis accounts for nearly two thirds of all nonobstetric exploratory laparotomies performed during pregnancy; most cases occur in the second and third trimesters.

The incidence of appendicitis is 0.4 to 1.4 per 1000 pregnancies.² Although the incidence of appendicitis in not increased during pregnancy, rupture of the appendix occurs 2 to 3 times more frequently in pregnancy secondary to delays in diagnosis and operation. Maternal and perinatal mortality and morbidity rates are greatly increased when appendicitis is complicated by peritonitis.

A difficult diagnosis

Diagnosis is difficult because many symptoms are considered to be normal during pregnancy. Many times, pain in the right lower quadrant of the abdomen may be attributed to round ligament pain or urinary tract infection. After the first trimester, the appendix is gradually displaced above McBurney’s point, with horizontal rotation of its base. This upward displacement occurs until the eighth month of gestation, when more than 90% of appendices lie above the iliac crest, and 80% rotate upward and toward the right subcostal area.^2,3

The most consistent clinical symptom encountered in pregnant women with appendicitis is vague right-sided abdominal pain.² Depending on the gestation, muscle guarding and rebound tenderness may or may not be present. Nausea, vomiting, and anorexia are usually present as in the nonpregnant patient. Twenty five percent of pregnant patients with appendicitis are afebrile, as our patient was.^2,4

The leukocytosis of pregnancy makes it difficult to determine if there is an infection. Not all pregnant patients with appendicitis will have a white blood cell count greater than 16,000/μL, but approximately 75% of them will have a left shift in the differential.² A urinalysis may reveal pyuria and hematuria and can mislead the physician to explain the symptoms as pyelonephritis.²

Treatment: Appendectomy, antibiotics if needed

Treatment of nonperforated acute appendicitis in pregnancy is appendectomy. In the first trimester, a laparoscopic appendectomy may be performed.² Intravenous antibiotics are indicated with perforation, peritonitis or abscess formation.^2,5

Tocolysis is unnecessary in uncomplicated appendicitis, but may be indicated if the patient goes into labor after surgery. In the late third trimester, with perforation or peritonitis, a cesarean section is indicated.

Evaluation is imperative

Fetal loss may occur in association with preterm labor and delivery or with generalized peritonitis and sepsis, and occurs only rarely in uncomplicated appendicitis. Fetal loss appears to be more closely associated with severity of appendicitis than with surgical intervention.^2,5,6

Imaging test characteristics: Is sonography enough?

Thus, it is imperative that any pregnant patient that comes in to the hospital or clinic with abdominal pain be evaluated for appendicitis. Ultrasound was a valuable diagnostic tool in this case and saved both the patient and developing fetus the radiation exposure of a CT scan. Ultrasound has a high specificity for diagnosing appendicitis if the appendix is visualized with abnormal findings. However, the sensitivity is not as high as CT, and failure to visualize the appendix adequately would have required a decision between appendectomy on clinical grounds only or going through with the CT scan.

The sensitivity, specificity, and positive and negative predictive values for ultrasonography and CT scans in the diagnosis of appendicitis are given in the TABLE (level of evidence [LOE]=1a).⁷

In a prospective study of patients with clinical signs and symptoms of acute appendicitis using a graded compression technique of ultrasonography, sonographic testing was as accurate as the focused unenhanced single-detector helical CT. The primary sonographic criterion for diagnosing acute appendicitis was an incompressible appendix with a transverse outer diameter of 6 mm or larger, as seen in this patient. The sensitivity of CT and sonography was 76% and 79%, respectively; the specificity was 83% and 78%; the accuracy was 78% and 78%; the positive predictive value was 90% and 87%; and the negative predictive value was 64% and 65% (LOE=2a).⁸

In conclusion, it is reasonable to use graded compression ultrasonography in a pregnant woman with suspected appendicitis. If the suspicion for appendicitis is high, a negative result may still need further evaluation with a CT or ultimately lead to abdominal surgery despite negative imaging studies.

TABLEUltrasound and CT in the diagnosis of appendicitis

Corresponding Author
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 21-year-old woman came to the clinic, frightened by a painful purpuric rash on her lower extremities (FIGURES 1 AND 2). The lesions appeared suddenly 3 days before, with no prior similar episodes.

The pain, and some swelling that happened when she stood, had finally driven her to take some time off from her job and seek medical advice. She was diagnosed with a case of pharyngitis earlier that week; due to multiple drug allergies, she was prescribed a course of clindamycin. She had not experienced any nausea or vomiting, fever, abdominal cramping, or gross hematuria.

On examination, the patient was friendly and good-humored, although she was concerned about her rash and visibly uncomfortable. She was walking with the aid of a borrowed cane, but her lesions were no longer tender to palpation.

The rash consisted mainly of purpuric papules almost entirely limited to her legs, although some isolated lesions were on her back as well. The papules were concentrated around her distal lower extremities, with a clear line of lesions encircling her calves bilaterally where her knee-high socks had applied pressure for the last 2 days (FIGURE 3) Mild edema was noted, but the rest of her physical exam was normal. By dip-stick, the patient had blood in her urine but no protein.

FIGURE 1
Purpura on the lower leg

FIGURE 2
Close-up

FIGURE 3
Visible sock lines

What is the diagnosis?
What is the treatment for this condition?

Diagnosis: Henoch-Schönlein purpura

This patient has Henoch-Schönlein purpura (HSP), a systemic vasculitis, secondary to hypersensitivity, occurring most commonly in children and young adults. Its classic triad includes palpable purpura, abdominal pain or renal involvement, and arthritis.¹

Purpura is typically nonblanching, as it represents extravasation of blood into skin, ² but lesions may also include urticarial wheals or occasional target lesions. As seen in this patient, the lower extremities and buttocks are typically affected. This rash is pressure- and gravity-dependent and pressure lines may develop, causing “sock lines” such as those seen on this young woman’s calves.^1,2 Arthritis also tends to affect large joints in lower extremities more severely than upper extremities, and ankles and knees may be swollen and tender, as observed in our patient.

Abdominal pain, not present in this case, develops in up to 65% of cases, and may be accompanied by vomiting, hematemesis, or blood in stools.

Renal involvement is by far the most long-reaching and potentially serious complication of HSP, particularly in adults. Although only 1% of all HSP patients may develop end-stage renal failure, it is estimated that in adults, this number may be as high as 11%.^3,4 The young woman in question presented with microscopic hematuria initially, which had resolved by the time of her next visit, 4 days later. A worse prognosis is associated with those patients who exhibit both nephritic and nephrotic features, with hematuria and proteinuria, where clinical remission has been shown to present only in 20% of these patients. Finally, as renal involvement may develop up to years after initial diagnosis, follow-up is crucial to monitor renal function.⁴

Cause is unknown

The cause of HSP is unknown, although it is thought to be secondary to deposition of immune complexes, and has been associated with vaccinations, viral infections, allergens in foods, drug reactions, exposure to cold, and bacterial respiratory infections, particularly involving group A streptococci.^1,2 This patient had been diagnosed with streptococcal pharyngitis and had been given a course of clindamycin due to her numerous drug allergies. Although the precise cause of her HSP is not definite, drugs have been shown to cause approximately 10% of acute cutaneous vasculitis cases, and she had never before been given clindamycin, possibly pointing to the origin of her disease.⁵

Differential diagnoses

Differential diagnoses for HSP depend on the level of specificity with which the triad presents. When symptoms are not typical, abdominal cramping alone may be confused with acute abdomen. Rash may be predominantly in the form of target lesions, and can be mistaken for erythema multiforme, particularly after a suspected drug reaction. Rheumatoid arthritis or systemic lupus erythematosus may be suspected with arthralgias. Idiopathic thrombocytopenic purpura can be distinguished from HSP by platelet count. Trauma or meningococcal septicemia may also be mistaken for the correct diagnosis in the presence of an atypical rash with few accompanying symptoms.

Treatment: Steroids, NSAIDs, and careful monitoring of renal function

Corticosteroids may be administered for severe abdominal symptoms, but they have not been shown to be useful in the absence of such symptoms. Conservative management with NSAIDs is the treatment of choice, and careful monitoring of renal function, even in the absence of renal involvement.^1,3,5

Outcome and discussion

Due to the presence of the classic HSP triad in our patient, we deemed a biopsy necessary, and managed the patient with ibupro-fen. One week later, her rash had receded and her microscopic hematuria was gone, but the vasculitis had led to lower-extremity edema. The patient’s feet were painfully swollen, and where the patient had scratched a lesion, her foot exhibited honey crusting and warmth, indicating a probable infection (FIGURES 4 AND 5). Her feet and ankles were so swollen and painful that she had difficulty walking. She was given cephalexin 500 mg 4 times daily for her infection, and was also restarted on oral prednisone at 60 mg daily until she could see the rheumatologist 2 days later. She was also issued crutches.

The rheumatologist agreed that this was HSP and told her that she would need to continue the prednisone for 2 months due to the severity of her cutaneous vasculitis. Within 5 days, the patient’s rash had diminished significantly. She was able to walk with less pain, as her ankle swelling decreased (FIGURE 6).

FIGURE 4
Swollen ankle

FIGURE 5
Probable infection

FIGURE 6
The same patient after 5 days

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Sciences Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 21-year-old woman came to the clinic, frightened by a painful purpuric rash on her lower extremities (FIGURES 1 AND 2). The lesions appeared suddenly 3 days before, with no prior similar episodes.

The pain, and some swelling that happened when she stood, had finally driven her to take some time off from her job and seek medical advice. She was diagnosed with a case of pharyngitis earlier that week; due to multiple drug allergies, she was prescribed a course of clindamycin. She had not experienced any nausea or vomiting, fever, abdominal cramping, or gross hematuria.

On examination, the patient was friendly and good-humored, although she was concerned about her rash and visibly uncomfortable. She was walking with the aid of a borrowed cane, but her lesions were no longer tender to palpation.

The rash consisted mainly of purpuric papules almost entirely limited to her legs, although some isolated lesions were on her back as well. The papules were concentrated around her distal lower extremities, with a clear line of lesions encircling her calves bilaterally where her knee-high socks had applied pressure for the last 2 days (FIGURE 3) Mild edema was noted, but the rest of her physical exam was normal. By dip-stick, the patient had blood in her urine but no protein.

FIGURE 1
Purpura on the lower leg

FIGURE 2
Close-up

FIGURE 3
Visible sock lines

What is the diagnosis?
What is the treatment for this condition?

Diagnosis: Henoch-Schönlein purpura

This patient has Henoch-Schönlein purpura (HSP), a systemic vasculitis, secondary to hypersensitivity, occurring most commonly in children and young adults. Its classic triad includes palpable purpura, abdominal pain or renal involvement, and arthritis.¹

Purpura is typically nonblanching, as it represents extravasation of blood into skin, ² but lesions may also include urticarial wheals or occasional target lesions. As seen in this patient, the lower extremities and buttocks are typically affected. This rash is pressure- and gravity-dependent and pressure lines may develop, causing “sock lines” such as those seen on this young woman’s calves.^1,2 Arthritis also tends to affect large joints in lower extremities more severely than upper extremities, and ankles and knees may be swollen and tender, as observed in our patient.

Abdominal pain, not present in this case, develops in up to 65% of cases, and may be accompanied by vomiting, hematemesis, or blood in stools.

Renal involvement is by far the most long-reaching and potentially serious complication of HSP, particularly in adults. Although only 1% of all HSP patients may develop end-stage renal failure, it is estimated that in adults, this number may be as high as 11%.^3,4 The young woman in question presented with microscopic hematuria initially, which had resolved by the time of her next visit, 4 days later. A worse prognosis is associated with those patients who exhibit both nephritic and nephrotic features, with hematuria and proteinuria, where clinical remission has been shown to present only in 20% of these patients. Finally, as renal involvement may develop up to years after initial diagnosis, follow-up is crucial to monitor renal function.⁴

Cause is unknown

The cause of HSP is unknown, although it is thought to be secondary to deposition of immune complexes, and has been associated with vaccinations, viral infections, allergens in foods, drug reactions, exposure to cold, and bacterial respiratory infections, particularly involving group A streptococci.^1,2 This patient had been diagnosed with streptococcal pharyngitis and had been given a course of clindamycin due to her numerous drug allergies. Although the precise cause of her HSP is not definite, drugs have been shown to cause approximately 10% of acute cutaneous vasculitis cases, and she had never before been given clindamycin, possibly pointing to the origin of her disease.⁵

Differential diagnoses

Differential diagnoses for HSP depend on the level of specificity with which the triad presents. When symptoms are not typical, abdominal cramping alone may be confused with acute abdomen. Rash may be predominantly in the form of target lesions, and can be mistaken for erythema multiforme, particularly after a suspected drug reaction. Rheumatoid arthritis or systemic lupus erythematosus may be suspected with arthralgias. Idiopathic thrombocytopenic purpura can be distinguished from HSP by platelet count. Trauma or meningococcal septicemia may also be mistaken for the correct diagnosis in the presence of an atypical rash with few accompanying symptoms.

Treatment: Steroids, NSAIDs, and careful monitoring of renal function

Corticosteroids may be administered for severe abdominal symptoms, but they have not been shown to be useful in the absence of such symptoms. Conservative management with NSAIDs is the treatment of choice, and careful monitoring of renal function, even in the absence of renal involvement.^1,3,5

Outcome and discussion

Due to the presence of the classic HSP triad in our patient, we deemed a biopsy necessary, and managed the patient with ibupro-fen. One week later, her rash had receded and her microscopic hematuria was gone, but the vasculitis had led to lower-extremity edema. The patient’s feet were painfully swollen, and where the patient had scratched a lesion, her foot exhibited honey crusting and warmth, indicating a probable infection (FIGURES 4 AND 5). Her feet and ankles were so swollen and painful that she had difficulty walking. She was given cephalexin 500 mg 4 times daily for her infection, and was also restarted on oral prednisone at 60 mg daily until she could see the rheumatologist 2 days later. She was also issued crutches.

The rheumatologist agreed that this was HSP and told her that she would need to continue the prednisone for 2 months due to the severity of her cutaneous vasculitis. Within 5 days, the patient’s rash had diminished significantly. She was able to walk with less pain, as her ankle swelling decreased (FIGURE 6).

FIGURE 4
Swollen ankle

FIGURE 5
Probable infection

FIGURE 6
The same patient after 5 days

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Sciences Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 21-year-old woman came to the clinic, frightened by a painful purpuric rash on her lower extremities (FIGURES 1 AND 2). The lesions appeared suddenly 3 days before, with no prior similar episodes.

The pain, and some swelling that happened when she stood, had finally driven her to take some time off from her job and seek medical advice. She was diagnosed with a case of pharyngitis earlier that week; due to multiple drug allergies, she was prescribed a course of clindamycin. She had not experienced any nausea or vomiting, fever, abdominal cramping, or gross hematuria.

On examination, the patient was friendly and good-humored, although she was concerned about her rash and visibly uncomfortable. She was walking with the aid of a borrowed cane, but her lesions were no longer tender to palpation.

The rash consisted mainly of purpuric papules almost entirely limited to her legs, although some isolated lesions were on her back as well. The papules were concentrated around her distal lower extremities, with a clear line of lesions encircling her calves bilaterally where her knee-high socks had applied pressure for the last 2 days (FIGURE 3) Mild edema was noted, but the rest of her physical exam was normal. By dip-stick, the patient had blood in her urine but no protein.

FIGURE 1
Purpura on the lower leg

FIGURE 2
Close-up

FIGURE 3
Visible sock lines

What is the diagnosis?
What is the treatment for this condition?

Diagnosis: Henoch-Schönlein purpura

This patient has Henoch-Schönlein purpura (HSP), a systemic vasculitis, secondary to hypersensitivity, occurring most commonly in children and young adults. Its classic triad includes palpable purpura, abdominal pain or renal involvement, and arthritis.¹

Purpura is typically nonblanching, as it represents extravasation of blood into skin, ² but lesions may also include urticarial wheals or occasional target lesions. As seen in this patient, the lower extremities and buttocks are typically affected. This rash is pressure- and gravity-dependent and pressure lines may develop, causing “sock lines” such as those seen on this young woman’s calves.^1,2 Arthritis also tends to affect large joints in lower extremities more severely than upper extremities, and ankles and knees may be swollen and tender, as observed in our patient.

Abdominal pain, not present in this case, develops in up to 65% of cases, and may be accompanied by vomiting, hematemesis, or blood in stools.

Renal involvement is by far the most long-reaching and potentially serious complication of HSP, particularly in adults. Although only 1% of all HSP patients may develop end-stage renal failure, it is estimated that in adults, this number may be as high as 11%.^3,4 The young woman in question presented with microscopic hematuria initially, which had resolved by the time of her next visit, 4 days later. A worse prognosis is associated with those patients who exhibit both nephritic and nephrotic features, with hematuria and proteinuria, where clinical remission has been shown to present only in 20% of these patients. Finally, as renal involvement may develop up to years after initial diagnosis, follow-up is crucial to monitor renal function.⁴

Cause is unknown

The cause of HSP is unknown, although it is thought to be secondary to deposition of immune complexes, and has been associated with vaccinations, viral infections, allergens in foods, drug reactions, exposure to cold, and bacterial respiratory infections, particularly involving group A streptococci.^1,2 This patient had been diagnosed with streptococcal pharyngitis and had been given a course of clindamycin due to her numerous drug allergies. Although the precise cause of her HSP is not definite, drugs have been shown to cause approximately 10% of acute cutaneous vasculitis cases, and she had never before been given clindamycin, possibly pointing to the origin of her disease.⁵

Differential diagnoses

Differential diagnoses for HSP depend on the level of specificity with which the triad presents. When symptoms are not typical, abdominal cramping alone may be confused with acute abdomen. Rash may be predominantly in the form of target lesions, and can be mistaken for erythema multiforme, particularly after a suspected drug reaction. Rheumatoid arthritis or systemic lupus erythematosus may be suspected with arthralgias. Idiopathic thrombocytopenic purpura can be distinguished from HSP by platelet count. Trauma or meningococcal septicemia may also be mistaken for the correct diagnosis in the presence of an atypical rash with few accompanying symptoms.

Treatment: Steroids, NSAIDs, and careful monitoring of renal function

Corticosteroids may be administered for severe abdominal symptoms, but they have not been shown to be useful in the absence of such symptoms. Conservative management with NSAIDs is the treatment of choice, and careful monitoring of renal function, even in the absence of renal involvement.^1,3,5

Outcome and discussion

Due to the presence of the classic HSP triad in our patient, we deemed a biopsy necessary, and managed the patient with ibupro-fen. One week later, her rash had receded and her microscopic hematuria was gone, but the vasculitis had led to lower-extremity edema. The patient’s feet were painfully swollen, and where the patient had scratched a lesion, her foot exhibited honey crusting and warmth, indicating a probable infection (FIGURES 4 AND 5). Her feet and ankles were so swollen and painful that she had difficulty walking. She was given cephalexin 500 mg 4 times daily for her infection, and was also restarted on oral prednisone at 60 mg daily until she could see the rheumatologist 2 days later. She was also issued crutches.

The rheumatologist agreed that this was HSP and told her that she would need to continue the prednisone for 2 months due to the severity of her cutaneous vasculitis. Within 5 days, the patient’s rash had diminished significantly. She was able to walk with less pain, as her ankle swelling decreased (FIGURE 6).

FIGURE 4
Swollen ankle

FIGURE 5
Probable infection

FIGURE 6
The same patient after 5 days

CORRESPONDING AUTHOR
Richard P. Usatine, MD, University of Texas Health Sciences Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 57-year-old retired farm worker came into the clinic with itchy pink rings all over his abdomen, legs, and arms. He said that the rash began 13 years ago, soon after he had retired from working in the fields. He said he had been exposed to pesticide chemicals but no other toxins. He’d never received a diagnosis of atopy or “sensitive skin.” He had sun damage on his lower arms, but none on the areas where the rash appeared.

Over the years, he had tried over-the-counter topical steroids, antifungals, and oral antihistamines, all with minimal relief. Furthermore, the lesions did not respond to 2 courses of oral antifungal medications. He said, “some days it itches more than others, but I just deal with it.” The patient was otherwise healthy and on no medications. He had been treated for active tuberculosis in the past but had no symptoms at this time. Out of his frustration, he recently began using paint thinner on the lesions. He thought the paint thinner “dried the rash out” and decreased the itching.

The distribution of the lesions can be seen in Figure 1. Erythematous rings can be seen on the abdomen and legs. These vary in size and shape. Figure 2 is a close-up of the left thigh showing the scale. There was no blanching erythema, no induration, no warmth, and no tenderness to palpation.

FIGURE 1
Rings on the trunk and legs

FIGURE 2
Close-up of thigh

What is the Diagnosis?

Diagnosis: Erythema Annulare Centrifugum

This patient has a rare skin condition called erythema annulare centrifugum (EAC). The primary lesion begins as a group of pink/erythematous papules that spread peripherally as they clear centrally, forming a “trailing scale” (Figure 3) They can enlarge at a rate of 2 to 5 mm/d, producing a plaque or patch of annular, arcuate, or polycyclic shape.¹ The margin is indurated and can vary in width from 4 to 6 mm. Vesicles may also be present. Distribution is usually on the thighs and legs but can also appear on the upper extremities (Figure 4), trunk, or face.

Differential diagnosis

Other causes of similar erythematous itchy rings to consider include pityriasis rosea, tinea corporis, psoriasis, nummular eczema, atopic dermatitis, drug reaction, and erythema migrans. The physical exam is the most effective method to differentiate between the above diagnoses—specifically, the distribution and morphology of the lesions. The natural history of the lesions can further narrow the diagnosis.

Pityriasis rosea has erythematous patches distributed on the trunk and extremities. These patches have a distinctive collarette border. The “herald patch,” the first patch to appear, is often oval and about 1 to 3 inches in diameter. Lesions classically follow skin lines to create a “Christmas tree” pattern on the back. Unlike EAC, pityriasis only lasts 6 to 8 weeks.

Tinea corporis (ringworm) is a fungal infection that is often a single scaly annular patch. Like EAC, it can be erythematous and is distributed anywhere on the body. When it presents as multiple lesions, it more closely resembles EAC. Unlike EAC, however, it should show branched hyphae with septae on a KOH prep and fungal culture would show growth of dermatophytes. Furthermore, it responds well to 4 to 6 weeks of antifungal treatment.

Psoriasis may present as plaques on the extensor surfaces of extremities. “Guttate” psoriasis (meaning “like water drops”) appears like small drops on the back and abdomen. Psoriatic plaques do not have central clearing or the trailing scale characteristic of EAC. Psoriatic plaques are often more responsive to steroids than EAC.

FIGURE 3
“Trailing scale”on abdomen

FIGURE 4
Ring on inner arm

Epidemiology and Pathophysiology

EAC is an uncommon inflammatory skin condition of unknown cause. Histologically, it resembles pityriasis rosea, having a superficial perivascular lymphocytic infiltrate with moderate epidermal acanthosis.^1,2 The primary lesions spread peripherally and clear centrally at a maximum rate of 2 to 5 mm/d. Pruritus is common but not always present.¹

Although the cause and exact pathogenesis are unknown, EAC may in some cases be triggered by drugs, bacterial infections (like tuberculosis), fungi, viruses, parasites, autoimmune diseases, neoplasms, liver diseases, and dysproteinemias.^1,3 Other hypotheses suggest that it may be a type IV hypersensitivity reaction.¹ The mean duration is 11 months, but it can vary from as little as 4 weeks to 34 years.¹

Diagnostic Tests

No specific tests are needed to make the diagnosis of EAC. However, it is a good idea to do a KOH prep to rule out tinea corporis or candidiasis. This technique has a sensitivity of 88% and a specificity of 95% for tinea.⁴ Thus, a positive result is sufficient for diagnosis. Fungal culture may be useful if you believe that the diagnosis is truly fungal but the KOH result is negative.

If physical exam leads to suspicion of underlying disease, it is important to order the appropriate diagnostic lab tests. A few case reports have described EAC as the presenting sign in patients diagnosed with cancer approximately 2 years later.¹

Treatment

It is helpful if you can find an underlying disease process because most cases of EAC clear as the underlying disease is treated. Review the current drugs and consider replacing any drug reported to have been associated with EAC: chloroquine, hydroxychloroquine, estrogen, cimetidine, penicillin, salicylates, piroxicam, hydrochlorothiazide, and amitriptyline.¹

If you have minimal suspicion for an underlying disease process and no causative drug agent is found, most cases of EAC require no treatment and resolve spontaneously.¹ However, if pruritus is significant, treat symptomatically. One option is to start with a topical high-potency steroid ointment twice daily. Use this regimen for 2 weeks, then assess response. If the response is good, you may continue a mid-potency steroid as needed or pulse-dosing of a high-potency steroid on the weekends. The goal is to minimize the itching while also minimizing the side effects associated with long-term steroid treatment. Treat as needed and taper when possible. Steroids may cause resolution of lesions but do not prevent recurrence.

A second option is topical calcipotriol, a topical vitamin D derivative. A recent case report has shown this to be beneficial,² but this is an off-label indication. To date, no randomized clinical trials have been done to demonstrate the efficacy of any other treatment.

Patient Outcome

A KOH prep and a fungal culture were obtained to confidently rule out tinea. Both results were negative. We decided to order a chest x-ray because of his past history of active tuberculosis; the test result was negative. To investigate for any underlying diseases, we ordered a complete blood count, chemistry panel, and erythrocyte sedimentation rate, which all showed normal results.

We explained the diagnosis of EAC as a chronic condition of unknown cause. Since steroids did not provide any relief for him in the past, we offered the option of using calcipotriol ointment, even though the evidence is only based on 1 case report.² He chose to try the calcipotriol. We warned the patient to not use paint thinner because inhalation and skin exposure are harmful. The patient agreed to stop using it.

Corresponding author
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu.

A 57-year-old retired farm worker came into the clinic with itchy pink rings all over his abdomen, legs, and arms. He said that the rash began 13 years ago, soon after he had retired from working in the fields. He said he had been exposed to pesticide chemicals but no other toxins. He’d never received a diagnosis of atopy or “sensitive skin.” He had sun damage on his lower arms, but none on the areas where the rash appeared.

Over the years, he had tried over-the-counter topical steroids, antifungals, and oral antihistamines, all with minimal relief. Furthermore, the lesions did not respond to 2 courses of oral antifungal medications. He said, “some days it itches more than others, but I just deal with it.” The patient was otherwise healthy and on no medications. He had been treated for active tuberculosis in the past but had no symptoms at this time. Out of his frustration, he recently began using paint thinner on the lesions. He thought the paint thinner “dried the rash out” and decreased the itching.

The distribution of the lesions can be seen in Figure 1. Erythematous rings can be seen on the abdomen and legs. These vary in size and shape. Figure 2 is a close-up of the left thigh showing the scale. There was no blanching erythema, no induration, no warmth, and no tenderness to palpation.

FIGURE 1
Rings on the trunk and legs

FIGURE 2
Close-up of thigh

What is the Diagnosis?

Diagnosis: Erythema Annulare Centrifugum

This patient has a rare skin condition called erythema annulare centrifugum (EAC). The primary lesion begins as a group of pink/erythematous papules that spread peripherally as they clear centrally, forming a “trailing scale” (Figure 3) They can enlarge at a rate of 2 to 5 mm/d, producing a plaque or patch of annular, arcuate, or polycyclic shape.¹ The margin is indurated and can vary in width from 4 to 6 mm. Vesicles may also be present. Distribution is usually on the thighs and legs but can also appear on the upper extremities (Figure 4), trunk, or face.

Differential diagnosis

Other causes of similar erythematous itchy rings to consider include pityriasis rosea, tinea corporis, psoriasis, nummular eczema, atopic dermatitis, drug reaction, and erythema migrans. The physical exam is the most effective method to differentiate between the above diagnoses—specifically, the distribution and morphology of the lesions. The natural history of the lesions can further narrow the diagnosis.

Pityriasis rosea has erythematous patches distributed on the trunk and extremities. These patches have a distinctive collarette border. The “herald patch,” the first patch to appear, is often oval and about 1 to 3 inches in diameter. Lesions classically follow skin lines to create a “Christmas tree” pattern on the back. Unlike EAC, pityriasis only lasts 6 to 8 weeks.

Tinea corporis (ringworm) is a fungal infection that is often a single scaly annular patch. Like EAC, it can be erythematous and is distributed anywhere on the body. When it presents as multiple lesions, it more closely resembles EAC. Unlike EAC, however, it should show branched hyphae with septae on a KOH prep and fungal culture would show growth of dermatophytes. Furthermore, it responds well to 4 to 6 weeks of antifungal treatment.

Psoriasis may present as plaques on the extensor surfaces of extremities. “Guttate” psoriasis (meaning “like water drops”) appears like small drops on the back and abdomen. Psoriatic plaques do not have central clearing or the trailing scale characteristic of EAC. Psoriatic plaques are often more responsive to steroids than EAC.

FIGURE 3
“Trailing scale”on abdomen

FIGURE 4
Ring on inner arm

Epidemiology and Pathophysiology

EAC is an uncommon inflammatory skin condition of unknown cause. Histologically, it resembles pityriasis rosea, having a superficial perivascular lymphocytic infiltrate with moderate epidermal acanthosis.^1,2 The primary lesions spread peripherally and clear centrally at a maximum rate of 2 to 5 mm/d. Pruritus is common but not always present.¹

Although the cause and exact pathogenesis are unknown, EAC may in some cases be triggered by drugs, bacterial infections (like tuberculosis), fungi, viruses, parasites, autoimmune diseases, neoplasms, liver diseases, and dysproteinemias.^1,3 Other hypotheses suggest that it may be a type IV hypersensitivity reaction.¹ The mean duration is 11 months, but it can vary from as little as 4 weeks to 34 years.¹

Diagnostic Tests

No specific tests are needed to make the diagnosis of EAC. However, it is a good idea to do a KOH prep to rule out tinea corporis or candidiasis. This technique has a sensitivity of 88% and a specificity of 95% for tinea.⁴ Thus, a positive result is sufficient for diagnosis. Fungal culture may be useful if you believe that the diagnosis is truly fungal but the KOH result is negative.

If physical exam leads to suspicion of underlying disease, it is important to order the appropriate diagnostic lab tests. A few case reports have described EAC as the presenting sign in patients diagnosed with cancer approximately 2 years later.¹

Treatment

It is helpful if you can find an underlying disease process because most cases of EAC clear as the underlying disease is treated. Review the current drugs and consider replacing any drug reported to have been associated with EAC: chloroquine, hydroxychloroquine, estrogen, cimetidine, penicillin, salicylates, piroxicam, hydrochlorothiazide, and amitriptyline.¹

If you have minimal suspicion for an underlying disease process and no causative drug agent is found, most cases of EAC require no treatment and resolve spontaneously.¹ However, if pruritus is significant, treat symptomatically. One option is to start with a topical high-potency steroid ointment twice daily. Use this regimen for 2 weeks, then assess response. If the response is good, you may continue a mid-potency steroid as needed or pulse-dosing of a high-potency steroid on the weekends. The goal is to minimize the itching while also minimizing the side effects associated with long-term steroid treatment. Treat as needed and taper when possible. Steroids may cause resolution of lesions but do not prevent recurrence.

A second option is topical calcipotriol, a topical vitamin D derivative. A recent case report has shown this to be beneficial,² but this is an off-label indication. To date, no randomized clinical trials have been done to demonstrate the efficacy of any other treatment.

Patient Outcome

A KOH prep and a fungal culture were obtained to confidently rule out tinea. Both results were negative. We decided to order a chest x-ray because of his past history of active tuberculosis; the test result was negative. To investigate for any underlying diseases, we ordered a complete blood count, chemistry panel, and erythrocyte sedimentation rate, which all showed normal results.

We explained the diagnosis of EAC as a chronic condition of unknown cause. Since steroids did not provide any relief for him in the past, we offered the option of using calcipotriol ointment, even though the evidence is only based on 1 case report.² He chose to try the calcipotriol. We warned the patient to not use paint thinner because inhalation and skin exposure are harmful. The patient agreed to stop using it.

Corresponding author
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu.

A 57-year-old retired farm worker came into the clinic with itchy pink rings all over his abdomen, legs, and arms. He said that the rash began 13 years ago, soon after he had retired from working in the fields. He said he had been exposed to pesticide chemicals but no other toxins. He’d never received a diagnosis of atopy or “sensitive skin.” He had sun damage on his lower arms, but none on the areas where the rash appeared.

Over the years, he had tried over-the-counter topical steroids, antifungals, and oral antihistamines, all with minimal relief. Furthermore, the lesions did not respond to 2 courses of oral antifungal medications. He said, “some days it itches more than others, but I just deal with it.” The patient was otherwise healthy and on no medications. He had been treated for active tuberculosis in the past but had no symptoms at this time. Out of his frustration, he recently began using paint thinner on the lesions. He thought the paint thinner “dried the rash out” and decreased the itching.

The distribution of the lesions can be seen in Figure 1. Erythematous rings can be seen on the abdomen and legs. These vary in size and shape. Figure 2 is a close-up of the left thigh showing the scale. There was no blanching erythema, no induration, no warmth, and no tenderness to palpation.

FIGURE 1
Rings on the trunk and legs

FIGURE 2
Close-up of thigh

What is the Diagnosis?

Diagnosis: Erythema Annulare Centrifugum

This patient has a rare skin condition called erythema annulare centrifugum (EAC). The primary lesion begins as a group of pink/erythematous papules that spread peripherally as they clear centrally, forming a “trailing scale” (Figure 3) They can enlarge at a rate of 2 to 5 mm/d, producing a plaque or patch of annular, arcuate, or polycyclic shape.¹ The margin is indurated and can vary in width from 4 to 6 mm. Vesicles may also be present. Distribution is usually on the thighs and legs but can also appear on the upper extremities (Figure 4), trunk, or face.

Differential diagnosis

Other causes of similar erythematous itchy rings to consider include pityriasis rosea, tinea corporis, psoriasis, nummular eczema, atopic dermatitis, drug reaction, and erythema migrans. The physical exam is the most effective method to differentiate between the above diagnoses—specifically, the distribution and morphology of the lesions. The natural history of the lesions can further narrow the diagnosis.

Pityriasis rosea has erythematous patches distributed on the trunk and extremities. These patches have a distinctive collarette border. The “herald patch,” the first patch to appear, is often oval and about 1 to 3 inches in diameter. Lesions classically follow skin lines to create a “Christmas tree” pattern on the back. Unlike EAC, pityriasis only lasts 6 to 8 weeks.

Tinea corporis (ringworm) is a fungal infection that is often a single scaly annular patch. Like EAC, it can be erythematous and is distributed anywhere on the body. When it presents as multiple lesions, it more closely resembles EAC. Unlike EAC, however, it should show branched hyphae with septae on a KOH prep and fungal culture would show growth of dermatophytes. Furthermore, it responds well to 4 to 6 weeks of antifungal treatment.

Psoriasis may present as plaques on the extensor surfaces of extremities. “Guttate” psoriasis (meaning “like water drops”) appears like small drops on the back and abdomen. Psoriatic plaques do not have central clearing or the trailing scale characteristic of EAC. Psoriatic plaques are often more responsive to steroids than EAC.

FIGURE 3
“Trailing scale”on abdomen

FIGURE 4
Ring on inner arm

Epidemiology and Pathophysiology

EAC is an uncommon inflammatory skin condition of unknown cause. Histologically, it resembles pityriasis rosea, having a superficial perivascular lymphocytic infiltrate with moderate epidermal acanthosis.^1,2 The primary lesions spread peripherally and clear centrally at a maximum rate of 2 to 5 mm/d. Pruritus is common but not always present.¹

Although the cause and exact pathogenesis are unknown, EAC may in some cases be triggered by drugs, bacterial infections (like tuberculosis), fungi, viruses, parasites, autoimmune diseases, neoplasms, liver diseases, and dysproteinemias.^1,3 Other hypotheses suggest that it may be a type IV hypersensitivity reaction.¹ The mean duration is 11 months, but it can vary from as little as 4 weeks to 34 years.¹

Diagnostic Tests

No specific tests are needed to make the diagnosis of EAC. However, it is a good idea to do a KOH prep to rule out tinea corporis or candidiasis. This technique has a sensitivity of 88% and a specificity of 95% for tinea.⁴ Thus, a positive result is sufficient for diagnosis. Fungal culture may be useful if you believe that the diagnosis is truly fungal but the KOH result is negative.

If physical exam leads to suspicion of underlying disease, it is important to order the appropriate diagnostic lab tests. A few case reports have described EAC as the presenting sign in patients diagnosed with cancer approximately 2 years later.¹

Treatment

It is helpful if you can find an underlying disease process because most cases of EAC clear as the underlying disease is treated. Review the current drugs and consider replacing any drug reported to have been associated with EAC: chloroquine, hydroxychloroquine, estrogen, cimetidine, penicillin, salicylates, piroxicam, hydrochlorothiazide, and amitriptyline.¹

If you have minimal suspicion for an underlying disease process and no causative drug agent is found, most cases of EAC require no treatment and resolve spontaneously.¹ However, if pruritus is significant, treat symptomatically. One option is to start with a topical high-potency steroid ointment twice daily. Use this regimen for 2 weeks, then assess response. If the response is good, you may continue a mid-potency steroid as needed or pulse-dosing of a high-potency steroid on the weekends. The goal is to minimize the itching while also minimizing the side effects associated with long-term steroid treatment. Treat as needed and taper when possible. Steroids may cause resolution of lesions but do not prevent recurrence.

A second option is topical calcipotriol, a topical vitamin D derivative. A recent case report has shown this to be beneficial,² but this is an off-label indication. To date, no randomized clinical trials have been done to demonstrate the efficacy of any other treatment.

Patient Outcome

A KOH prep and a fungal culture were obtained to confidently rule out tinea. Both results were negative. We decided to order a chest x-ray because of his past history of active tuberculosis; the test result was negative. To investigate for any underlying diseases, we ordered a complete blood count, chemistry panel, and erythrocyte sedimentation rate, which all showed normal results.

We explained the diagnosis of EAC as a chronic condition of unknown cause. Since steroids did not provide any relief for him in the past, we offered the option of using calcipotriol ointment, even though the evidence is only based on 1 case report.² He chose to try the calcipotriol. We warned the patient to not use paint thinner because inhalation and skin exposure are harmful. The patient agreed to stop using it.

Corresponding author
Richard P. Usatine, MD, University of Texas Health Science Center at San Antonio, Department of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu.

A 22-year-old man came into the office concerned he may have warts on his penis. He believed the warts appeared about 3 months ago. He was single and did not have a sexual partner. He had been dating a woman for 1 year until he graduated from college 4 months ago. His sexual history was serial monogamy with 5 lifetime female sexual partners.

After some hesitation, he noted he slept with a woman one night following a graduation party. He admitted that they were both drunk and that he did not use a condom. He asked if this was how the condition could have developed.

He denied any history of sexually transmitted diseases (STDs) and the result of an HIV test was negative when he donated blood last year. He did not have urethral discharge or burning on urination. The patient had no other symptoms and no chronic illnesses. He generally had a healthy lifestyle, without drug and tobacco use. He said he used to drink at college parties, but rarely had a drink since starting work full-time.

On physical exam, the patient had no fever or lymphadenopathy. A genital exam (Figure 1) with the foreskin retracted revealed skin-colored papules on the shaft of the penis that were somewhat verrucous. The papules seemed to make a ring around the shaft just proximal to the corona of the glans. Closer inspection showed smaller pearly papules surrounding the glans on the corona (Figure 2).

FIGURE 1
Large verrucuous papules

FIGURE 2
Detail of the smaller papules

Are The Larger Verrucous Papules Really Warts?
What Are The Smaller Papules And do they need treatment?

Diagnosis: Condyloma Acuminata

The larger verrucous papules are genital warts, also known as condyloma acuminata. These are caused by the human papillomavirus (HPV) and are sexually transmitted. The patient most likely acquired these from his last unprotected sexual encounter, but he may have been infected earlier and the warts just became visible in the last 3 months. The differential diagnosis includes condyloma lata, the flat warts of secondary syphilis, but these are much less common.

The small pearly papules on the corona are not warts but a variation of the normal male anatomy, called pearly penile papules. The reason to recognize these papules is to reassure worried men that they are normal and to avoid performing any invasive treatments to remove them.

Laboratory Examination

All patients with any sexually transmitted disease should be tested for syphilis and HIV regardless of other risk factors.¹ In this case, testing for syphilis with either a rapid plasma reagin (RPR) or VDRL will also be helpful to rule out condyloma lata.

These genital warts do not need to be biopsied to make the diagnosis. No data support the use of type-specific HPV nucleic acid tests in the routine diagnosis or management of visible genital warts.¹

Treatment: Removal With Medication Or Surgery

The primary goal of treating visible genital warts is the removal of symptomatic warts.¹ Treatment can induce wart-free periods. Available therapies for genital warts may reduce, but probably do not eradicate, infectivity.¹ No evidence suggests any available treatment is superior to another, and no single treatment is ideal for all patients or all warts. The natural history of genital warts is benign, and the types of HPV that usually cause external genital warts (HPV 6 and 11) are not associated with cancer.

The Centers for Disease Control and Prevention (CDC) 2002 treatment guidelines¹ for STDs recommend the following options. Cost data are given in the Table.

TABLE
Self-administered medications for HPV

Patient-applied treatments

• Podofilox 0.5% solution or gel. Patients should apply podofilox solution with a cotton swab, or podofilox gel with a finger, to visible genital warts twice a day for 3 days, followed by 4 days of no therapy. This cycle may be repeated, as necessary, for up to 4 cycles. The health care provider may apply the initial treatment to demonstrate the proper application technique and identify which warts should be treated.
• Imiquimod 5% cream. Patients should apply imiquimod cream once daily at bedtime, 3 times a week for up to 16 weeks. The treatment area should be washed with soap and water 6 to 10 hours after the application.

Provider-administered treatments

• Cryotherapy with liquid nitrogen or cryoprobe. Repeat applications every 1 to 2 weeks.
• Podophyllin resin 10% to 25% in a compound tincture of benzoin. A small amount should be applied to each wart and allowed to air-dry. The treatment can be repeated weekly, if necessary. To avoid the possibility of complications associated with systemic absorption and toxicity, some specialists recommend that application be limited to ≤0.5 mL of podophyllin or an area of <10 cm² of warts per session. Some specialists suggest the preparation should be thoroughly washed off 1 to 4 hours after application to reduce local irritation. The safety of podophyllin during pregnancy has not been established.
• Trichloroacetic acid (TCA): a small amount should be applied only to warts and allowed to dry, at which time a white “frosting” develops (Figure 3). This treatment can be repeated weekly, if necessary.
• Surgical removal either by tangential scissor excision, tangential shave excision, curettage, or electrosurgery.

FIGURE 3
Treatment of genital warts

Keratinized vs nonkeratinized warts

In choosing the type of therapy for a patient, it is helpful to note that the soft, nonkeratinized warts respond well to the various forms of podophyllin and trichloroacetic acid. The more keratinized lesions, however, respond better to physical ablative methods such as cryotherapy, excision, or electrocautery. Imiquimod appears to work well for both types of lesions but is more effective for the nonkeratinized warts.

The softer, nonkeratinized warts are found more often on softer mucosa around the anus, under the foreskin, and around the female introitus. The firmer, more keratinized warts are found more often on more keratinized skin such as on the shaft of the penis.

Most options yield inadequate results

The reason for so many treatment options is in part due to the therapeutic inadequacy of any one of them. Cure rates are far less than optimal, and relapse rates are disappointing. While we have reasonable treatment guidelines from the CDC, studies on many of the treatment options are limited. There are few head-to-head studies of one method vs another.

One double-blind, randomized, multicenter, vehicle-controlled study² demonstrated that 0.5% podofilox gel was significantly better than vehicle gel for successfully eliminating and reducing the number and size of anogenital warts. In the intention-to-treat population, 37% treated with podofilox gel had complete clearing of the treated areas, compared with 2% who had clearing of warts with the vehicle gel after 4 weeks (P<.001; number needed to treat=3) (level of evidence [LOE]: 1b).

The best systematic review of genital wart treatment was published in 2004 in the International Journal of STD & AIDS.³ Imiquimod has proven effective in many studies, including 3 randomized controlled trials in which clearance rates were 37% to 52% after 8 to 16 weeks of treatment (LOE=1a).³ One study using imiquimod found that clearance rates were twofold higher for women than for men (72% and 33%, respectively).⁴ This is probably because genital warts in females are less keratinized than warts on the usual site in males, the penile shaft. Similarly, clearance rates with imiquimod seem to be higher in uncircumcised men (62%) than circumcised men (33%), probably related to the degree of keratinization.³

Recurrence rates for sole therapy with imiquimod (9%–19%) are substantially lower than for most other genital wart treatments, including podophyllotoxin.³ Imiquimod may even be effective in reducing wart recurrence rates when used as an adjunct to surgical treatment.³

The Patient’s Treatment: Cryotherapy, Imiquimod Cream

The treatment options were discussed with the patient. The patient decided to have cryotherapy performed in the office and was given a prescription for imiquimod cream to be used on any remaining warts. The normal pearly penile papules on the corona were left alone and the patient tolerated the cryotherapy to the real warts with an acceptable level of temporary discomfort.

A 22-year-old man came into the office concerned he may have warts on his penis. He believed the warts appeared about 3 months ago. He was single and did not have a sexual partner. He had been dating a woman for 1 year until he graduated from college 4 months ago. His sexual history was serial monogamy with 5 lifetime female sexual partners.

After some hesitation, he noted he slept with a woman one night following a graduation party. He admitted that they were both drunk and that he did not use a condom. He asked if this was how the condition could have developed.

He denied any history of sexually transmitted diseases (STDs) and the result of an HIV test was negative when he donated blood last year. He did not have urethral discharge or burning on urination. The patient had no other symptoms and no chronic illnesses. He generally had a healthy lifestyle, without drug and tobacco use. He said he used to drink at college parties, but rarely had a drink since starting work full-time.

On physical exam, the patient had no fever or lymphadenopathy. A genital exam (Figure 1) with the foreskin retracted revealed skin-colored papules on the shaft of the penis that were somewhat verrucous. The papules seemed to make a ring around the shaft just proximal to the corona of the glans. Closer inspection showed smaller pearly papules surrounding the glans on the corona (Figure 2).

FIGURE 1
Large verrucuous papules

FIGURE 2
Detail of the smaller papules

Are The Larger Verrucous Papules Really Warts?
What Are The Smaller Papules And do they need treatment?

Diagnosis: Condyloma Acuminata

The larger verrucous papules are genital warts, also known as condyloma acuminata. These are caused by the human papillomavirus (HPV) and are sexually transmitted. The patient most likely acquired these from his last unprotected sexual encounter, but he may have been infected earlier and the warts just became visible in the last 3 months. The differential diagnosis includes condyloma lata, the flat warts of secondary syphilis, but these are much less common.

The small pearly papules on the corona are not warts but a variation of the normal male anatomy, called pearly penile papules. The reason to recognize these papules is to reassure worried men that they are normal and to avoid performing any invasive treatments to remove them.

Laboratory Examination

All patients with any sexually transmitted disease should be tested for syphilis and HIV regardless of other risk factors.¹ In this case, testing for syphilis with either a rapid plasma reagin (RPR) or VDRL will also be helpful to rule out condyloma lata.

These genital warts do not need to be biopsied to make the diagnosis. No data support the use of type-specific HPV nucleic acid tests in the routine diagnosis or management of visible genital warts.¹

Treatment: Removal With Medication Or Surgery

The primary goal of treating visible genital warts is the removal of symptomatic warts.¹ Treatment can induce wart-free periods. Available therapies for genital warts may reduce, but probably do not eradicate, infectivity.¹ No evidence suggests any available treatment is superior to another, and no single treatment is ideal for all patients or all warts. The natural history of genital warts is benign, and the types of HPV that usually cause external genital warts (HPV 6 and 11) are not associated with cancer.

The Centers for Disease Control and Prevention (CDC) 2002 treatment guidelines¹ for STDs recommend the following options. Cost data are given in the Table.

TABLE
Self-administered medications for HPV

Patient-applied treatments

• Podofilox 0.5% solution or gel. Patients should apply podofilox solution with a cotton swab, or podofilox gel with a finger, to visible genital warts twice a day for 3 days, followed by 4 days of no therapy. This cycle may be repeated, as necessary, for up to 4 cycles. The health care provider may apply the initial treatment to demonstrate the proper application technique and identify which warts should be treated.
• Imiquimod 5% cream. Patients should apply imiquimod cream once daily at bedtime, 3 times a week for up to 16 weeks. The treatment area should be washed with soap and water 6 to 10 hours after the application.

Provider-administered treatments

• Cryotherapy with liquid nitrogen or cryoprobe. Repeat applications every 1 to 2 weeks.
• Podophyllin resin 10% to 25% in a compound tincture of benzoin. A small amount should be applied to each wart and allowed to air-dry. The treatment can be repeated weekly, if necessary. To avoid the possibility of complications associated with systemic absorption and toxicity, some specialists recommend that application be limited to ≤0.5 mL of podophyllin or an area of <10 cm² of warts per session. Some specialists suggest the preparation should be thoroughly washed off 1 to 4 hours after application to reduce local irritation. The safety of podophyllin during pregnancy has not been established.
• Trichloroacetic acid (TCA): a small amount should be applied only to warts and allowed to dry, at which time a white “frosting” develops (Figure 3). This treatment can be repeated weekly, if necessary.
• Surgical removal either by tangential scissor excision, tangential shave excision, curettage, or electrosurgery.

FIGURE 3
Treatment of genital warts

Keratinized vs nonkeratinized warts

In choosing the type of therapy for a patient, it is helpful to note that the soft, nonkeratinized warts respond well to the various forms of podophyllin and trichloroacetic acid. The more keratinized lesions, however, respond better to physical ablative methods such as cryotherapy, excision, or electrocautery. Imiquimod appears to work well for both types of lesions but is more effective for the nonkeratinized warts.

The softer, nonkeratinized warts are found more often on softer mucosa around the anus, under the foreskin, and around the female introitus. The firmer, more keratinized warts are found more often on more keratinized skin such as on the shaft of the penis.

Most options yield inadequate results

The reason for so many treatment options is in part due to the therapeutic inadequacy of any one of them. Cure rates are far less than optimal, and relapse rates are disappointing. While we have reasonable treatment guidelines from the CDC, studies on many of the treatment options are limited. There are few head-to-head studies of one method vs another.

One double-blind, randomized, multicenter, vehicle-controlled study² demonstrated that 0.5% podofilox gel was significantly better than vehicle gel for successfully eliminating and reducing the number and size of anogenital warts. In the intention-to-treat population, 37% treated with podofilox gel had complete clearing of the treated areas, compared with 2% who had clearing of warts with the vehicle gel after 4 weeks (P<.001; number needed to treat=3) (level of evidence [LOE]: 1b).

The best systematic review of genital wart treatment was published in 2004 in the International Journal of STD & AIDS.³ Imiquimod has proven effective in many studies, including 3 randomized controlled trials in which clearance rates were 37% to 52% after 8 to 16 weeks of treatment (LOE=1a).³ One study using imiquimod found that clearance rates were twofold higher for women than for men (72% and 33%, respectively).⁴ This is probably because genital warts in females are less keratinized than warts on the usual site in males, the penile shaft. Similarly, clearance rates with imiquimod seem to be higher in uncircumcised men (62%) than circumcised men (33%), probably related to the degree of keratinization.³

Recurrence rates for sole therapy with imiquimod (9%–19%) are substantially lower than for most other genital wart treatments, including podophyllotoxin.³ Imiquimod may even be effective in reducing wart recurrence rates when used as an adjunct to surgical treatment.³

The Patient’s Treatment: Cryotherapy, Imiquimod Cream

The treatment options were discussed with the patient. The patient decided to have cryotherapy performed in the office and was given a prescription for imiquimod cream to be used on any remaining warts. The normal pearly penile papules on the corona were left alone and the patient tolerated the cryotherapy to the real warts with an acceptable level of temporary discomfort.

A 22-year-old man came into the office concerned he may have warts on his penis. He believed the warts appeared about 3 months ago. He was single and did not have a sexual partner. He had been dating a woman for 1 year until he graduated from college 4 months ago. His sexual history was serial monogamy with 5 lifetime female sexual partners.

After some hesitation, he noted he slept with a woman one night following a graduation party. He admitted that they were both drunk and that he did not use a condom. He asked if this was how the condition could have developed.

He denied any history of sexually transmitted diseases (STDs) and the result of an HIV test was negative when he donated blood last year. He did not have urethral discharge or burning on urination. The patient had no other symptoms and no chronic illnesses. He generally had a healthy lifestyle, without drug and tobacco use. He said he used to drink at college parties, but rarely had a drink since starting work full-time.

On physical exam, the patient had no fever or lymphadenopathy. A genital exam (Figure 1) with the foreskin retracted revealed skin-colored papules on the shaft of the penis that were somewhat verrucous. The papules seemed to make a ring around the shaft just proximal to the corona of the glans. Closer inspection showed smaller pearly papules surrounding the glans on the corona (Figure 2).

FIGURE 1
Large verrucuous papules

FIGURE 2
Detail of the smaller papules

Are The Larger Verrucous Papules Really Warts?
What Are The Smaller Papules And do they need treatment?

Diagnosis: Condyloma Acuminata

The larger verrucous papules are genital warts, also known as condyloma acuminata. These are caused by the human papillomavirus (HPV) and are sexually transmitted. The patient most likely acquired these from his last unprotected sexual encounter, but he may have been infected earlier and the warts just became visible in the last 3 months. The differential diagnosis includes condyloma lata, the flat warts of secondary syphilis, but these are much less common.

The small pearly papules on the corona are not warts but a variation of the normal male anatomy, called pearly penile papules. The reason to recognize these papules is to reassure worried men that they are normal and to avoid performing any invasive treatments to remove them.

Laboratory Examination

All patients with any sexually transmitted disease should be tested for syphilis and HIV regardless of other risk factors.¹ In this case, testing for syphilis with either a rapid plasma reagin (RPR) or VDRL will also be helpful to rule out condyloma lata.

These genital warts do not need to be biopsied to make the diagnosis. No data support the use of type-specific HPV nucleic acid tests in the routine diagnosis or management of visible genital warts.¹

Treatment: Removal With Medication Or Surgery

The primary goal of treating visible genital warts is the removal of symptomatic warts.¹ Treatment can induce wart-free periods. Available therapies for genital warts may reduce, but probably do not eradicate, infectivity.¹ No evidence suggests any available treatment is superior to another, and no single treatment is ideal for all patients or all warts. The natural history of genital warts is benign, and the types of HPV that usually cause external genital warts (HPV 6 and 11) are not associated with cancer.

The Centers for Disease Control and Prevention (CDC) 2002 treatment guidelines¹ for STDs recommend the following options. Cost data are given in the Table.

TABLE
Self-administered medications for HPV

Patient-applied treatments

• Podofilox 0.5% solution or gel. Patients should apply podofilox solution with a cotton swab, or podofilox gel with a finger, to visible genital warts twice a day for 3 days, followed by 4 days of no therapy. This cycle may be repeated, as necessary, for up to 4 cycles. The health care provider may apply the initial treatment to demonstrate the proper application technique and identify which warts should be treated.
• Imiquimod 5% cream. Patients should apply imiquimod cream once daily at bedtime, 3 times a week for up to 16 weeks. The treatment area should be washed with soap and water 6 to 10 hours after the application.

Provider-administered treatments

• Cryotherapy with liquid nitrogen or cryoprobe. Repeat applications every 1 to 2 weeks.
• Podophyllin resin 10% to 25% in a compound tincture of benzoin. A small amount should be applied to each wart and allowed to air-dry. The treatment can be repeated weekly, if necessary. To avoid the possibility of complications associated with systemic absorption and toxicity, some specialists recommend that application be limited to ≤0.5 mL of podophyllin or an area of <10 cm² of warts per session. Some specialists suggest the preparation should be thoroughly washed off 1 to 4 hours after application to reduce local irritation. The safety of podophyllin during pregnancy has not been established.
• Trichloroacetic acid (TCA): a small amount should be applied only to warts and allowed to dry, at which time a white “frosting” develops (Figure 3). This treatment can be repeated weekly, if necessary.
• Surgical removal either by tangential scissor excision, tangential shave excision, curettage, or electrosurgery.

FIGURE 3
Treatment of genital warts

Keratinized vs nonkeratinized warts

In choosing the type of therapy for a patient, it is helpful to note that the soft, nonkeratinized warts respond well to the various forms of podophyllin and trichloroacetic acid. The more keratinized lesions, however, respond better to physical ablative methods such as cryotherapy, excision, or electrocautery. Imiquimod appears to work well for both types of lesions but is more effective for the nonkeratinized warts.

The softer, nonkeratinized warts are found more often on softer mucosa around the anus, under the foreskin, and around the female introitus. The firmer, more keratinized warts are found more often on more keratinized skin such as on the shaft of the penis.

Most options yield inadequate results

The reason for so many treatment options is in part due to the therapeutic inadequacy of any one of them. Cure rates are far less than optimal, and relapse rates are disappointing. While we have reasonable treatment guidelines from the CDC, studies on many of the treatment options are limited. There are few head-to-head studies of one method vs another.

One double-blind, randomized, multicenter, vehicle-controlled study² demonstrated that 0.5% podofilox gel was significantly better than vehicle gel for successfully eliminating and reducing the number and size of anogenital warts. In the intention-to-treat population, 37% treated with podofilox gel had complete clearing of the treated areas, compared with 2% who had clearing of warts with the vehicle gel after 4 weeks (P<.001; number needed to treat=3) (level of evidence [LOE]: 1b).

The best systematic review of genital wart treatment was published in 2004 in the International Journal of STD & AIDS.³ Imiquimod has proven effective in many studies, including 3 randomized controlled trials in which clearance rates were 37% to 52% after 8 to 16 weeks of treatment (LOE=1a).³ One study using imiquimod found that clearance rates were twofold higher for women than for men (72% and 33%, respectively).⁴ This is probably because genital warts in females are less keratinized than warts on the usual site in males, the penile shaft. Similarly, clearance rates with imiquimod seem to be higher in uncircumcised men (62%) than circumcised men (33%), probably related to the degree of keratinization.³

Recurrence rates for sole therapy with imiquimod (9%–19%) are substantially lower than for most other genital wart treatments, including podophyllotoxin.³ Imiquimod may even be effective in reducing wart recurrence rates when used as an adjunct to surgical treatment.³

The Patient’s Treatment: Cryotherapy, Imiquimod Cream

The treatment options were discussed with the patient. The patient decided to have cryotherapy performed in the office and was given a prescription for imiquimod cream to be used on any remaining warts. The normal pearly penile papules on the corona were left alone and the patient tolerated the cryotherapy to the real warts with an acceptable level of temporary discomfort.

A 55-year-old woman came to the clinic complaining of severe itching on her arms and legs. Although she itched throughout the day, it became intolerable at night, disrupting her sleep. She would sometimes scratch her arms and legs until exhaustion but could find no relief. Being outside in warm and sunny weather aggravated the problem. She had used moisturizers, emollients, and topical corticosteroids, but they only alleviated the itching temporarily. The itching began 10 months earlier, just after she finalized the divorce from her husband of 20 years.

Examination of the skin revealed numerous excoriations with ulcerations and xerosis on the arms and left leg (Figure 1 and 2). The excoriations were located extensively from the dorsum of her left foot to above the knee and bilaterally from the wrist to above the elbow. They also showed signs of infection. The patient admitted they were self-inflicted. The patient’s right leg had been amputated 5 years before after a car accident, and she wore a prosthetic leg. Examination of other areas showed nothing remarkable.

The patient readily admitted to a great deal of psychological distress. She described feeling depressed since her divorce. She has had difficulty securing a full-time job and has high anxiety about being able to pay her rent and bills.

FIGURE 1
Excoriations on the arms…

FIGURE 2
…and the left leg

WHAT IS THE DIAGNOSIS?
WHAT IS THE TREATMENT STRATEGY?

DIFFERENTIAL DIAGNOSIS: PSYCHODERMATOLOGIC DISORDER

The patient’s history and physical examination points to a psychodermatologic disorder. Psycho-dermatologic disorders are conditions involving an interaction between the mind and skin and are classified as:³

• Psychophysiologic disorders:skin disorders worsened by emotional stress
• Primary psychiatric disorders:usually caused by psychological conditions with self-induced skin damage
• Secondary psychiatric disorders: psychological problems developed as a consequence of a disfiguring skin disorder, which negatively effects self-esteem and body image.³

This patient’s excoriations and ulcers are due to self-mutilation. The differential diagnosis includes psychogenic parasitosis, factitial dermatitis, and neurotic excoriations. These 3 skin conditions are primary psychiatric symptoms, and proper diagnosis revolves around being able to assess the dermatologic features and associated psychological disorder.²

Psychogenic parasitosis

Also known as delusional parasitosis, psychogenic parasitosis is a psychodermatologic disorder in which patients believe they are infested with parasites. Patients with this disorder report seeing or feeling parasites on their skin, and they damage their skin in an attempt to remove them. Patients create excoriations and ulcers on easily reached areas, usually the ears, eyes, nose, and extremities. They often present with what is termed the “matchbox sign,” in which they bring containers filled with “small bits of excoriated skin, dried blood, debris, or insect parts as proof of infestation.”²

Women over the age of 50 years are more often affected with psychogenic parasitosis, and the disorder is associated with anxiety, depression, and hypochondriasis.

Factitial dermatitis

Factitial dermatitis, also known as dermatitis artefacta, is a psychodermatologic disorder in which patients damage their skin but deny their self-involvement. This disorder encompasses a wide range of lesions, including blisters, cuts, ulcers, and burns. Patients often are unable to describe how the lesions evolved. Lesions exhibit bizarre patterns not characteristic of any disease.

Young adults and adolescents are more commonly affected, and it is 4 times more common among women than men. Psychological disorders involved with factitial dermatitis include personality disorders and posttraumatic stress disorder.²

Neurotic excoriations

Neurotic excoriations, sometimes referred to as neurodermatitis, are a result of a psychodermatologic disorder in which patients inflict excoriations and ulcers on their skin and admit to their involvement.³ The condition is characterized by excoriations similar in size and shape that are localized on areas easily reached by the patient, such as the arms, legs, and upper back.

The lesions may present in various stages, varying from dugout ulcers to ulcers covered with crusts and surrounded by erythema to areas receding into depressed scars. These lesions are a result of repetitive scratching and digging by the patient, usually without an underlying physical pathology but sometimes initiated by pruritus.^1,2

Studies show the condition primarily affects women, with a mean onset between the ages of 30 to 45 years.¹ Common psychiatric problems associated with neurotic excoriations include significant social stress, depression, anxiety, and obsessive-compulsive disorder.

Diagnosis: Neurotic excoriations due to depression and stress

The patient’s history and physical examination suggests a diagnosis of neurotic excoriations due to the characteristics of the excoriations and ulcers on her arms and leg, her admission of their self-inflicted nature, and the associated depression and psychosocial stress.

Laboratory tests

Although there are no available laboratory tests to confirm a positive finding of neurotic excoriations, tests could be performed to disprove any systemic causes of pruritus and the resulting dermatological damage.⁵ These tests include complete blood count with differential, chemistry profile, determination of thyroid-stimulating hormone levels, fasting plasma glucose level, and skin biopsies.^1,

Laboratory tests for systemic causes of pruritus should be based on the patient’s physical and history, avoiding a broad approach.

Treatment: address the skin and the psyche

The treatment of neurotic excoriations requires a dual approach, addressing both the dermatological problems and the underlying psychological disorder.³ Supportive dermatologic care is necessary to avoid secondary complications and to ensure that the patient feels supported.³

Dermatologic care

Topical steroids can be helpful to decrease the pruritus and inflammation. The steroid strength should be chosen based upon the severity of the lesions and the thickness of the skin. Steroid ointments are preferred to creams when there are skin ulcers and deep excoriations.

If there is significant crusting or exudate, there is probably a secondary bacterial infection and oral antibiotics are indicated ( Figure 3). A first-generation cephalosporin or dicloxacillin should provide adequate coverage against the most likely organisms, Staphylococcus aureus and Streptococcus pyogenes.

FIGURE 3
Detail of lesions on the arm

Psychological care

The selective serotonin reuptake inhibitors produce a strong antipruritic response in patients with neurotic excoriations. Doxepin is a tricyclic antidepressant with one of the most powerful antihistamines for pruritus. When prescribing doxepin, limit the amount of medication dispensed at one time to minimize the risk of suicide.³ The patient should trim the fingernails to reduce the amount of damage caused by scratching and digging.

Treating the underlying psychological disorder requires supportive and empathic counseling. It may be necessary to collaborate with other mental health specialists. Management options include psychotropic medication, stress management courses, and referral to a psychiatrist. Patients with psychodermatologic disorders frequently resist referral to mental health professionals.³ If this is the case, family physicians are well positioned to help patients with psychological problems.

Alternative courses of treatment include hypnosis to disrupt the itch-scratch cycle, and acupuncture and supportive therapy to reduce underlying stressors.¹

Patient’s outcome

By showing support and concern for the patient’s health during the visit, the physician strengthened the relationship with the patient, who felt comfortable disclosing many of her concerns and troubles. She received prescriptions for cephalexin 500 mg orally 3 times daily, 0.1% triamcinolone ointment to be applied twice daily, and doxepin 25 mg once nightly. She also received a referral for counseling.

At first she refused to trim her fingernails, but as she began to see that her ulcers were self-inflicted, she reconsidered and agreed. In fact, she asked to borrow our nail clippers before leaving the office.

Corresponding author
Richard P. Usatine, MD, University of Texas Health Sceicnes Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900.

Note: A handout developed by the American Academy of Family Physicians for patients with neurotic excoriations is available to print or photocopy at the following website: www.aafp.org/afp/20011215/neurph.html.

A 55-year-old woman came to the clinic complaining of severe itching on her arms and legs. Although she itched throughout the day, it became intolerable at night, disrupting her sleep. She would sometimes scratch her arms and legs until exhaustion but could find no relief. Being outside in warm and sunny weather aggravated the problem. She had used moisturizers, emollients, and topical corticosteroids, but they only alleviated the itching temporarily. The itching began 10 months earlier, just after she finalized the divorce from her husband of 20 years.

Examination of the skin revealed numerous excoriations with ulcerations and xerosis on the arms and left leg (Figure 1 and 2). The excoriations were located extensively from the dorsum of her left foot to above the knee and bilaterally from the wrist to above the elbow. They also showed signs of infection. The patient admitted they were self-inflicted. The patient’s right leg had been amputated 5 years before after a car accident, and she wore a prosthetic leg. Examination of other areas showed nothing remarkable.

The patient readily admitted to a great deal of psychological distress. She described feeling depressed since her divorce. She has had difficulty securing a full-time job and has high anxiety about being able to pay her rent and bills.

FIGURE 1
Excoriations on the arms…

FIGURE 2
…and the left leg

WHAT IS THE DIAGNOSIS?
WHAT IS THE TREATMENT STRATEGY?

DIFFERENTIAL DIAGNOSIS: PSYCHODERMATOLOGIC DISORDER

The patient’s history and physical examination points to a psychodermatologic disorder. Psycho-dermatologic disorders are conditions involving an interaction between the mind and skin and are classified as:³

• Psychophysiologic disorders:skin disorders worsened by emotional stress
• Primary psychiatric disorders:usually caused by psychological conditions with self-induced skin damage
• Secondary psychiatric disorders: psychological problems developed as a consequence of a disfiguring skin disorder, which negatively effects self-esteem and body image.³

This patient’s excoriations and ulcers are due to self-mutilation. The differential diagnosis includes psychogenic parasitosis, factitial dermatitis, and neurotic excoriations. These 3 skin conditions are primary psychiatric symptoms, and proper diagnosis revolves around being able to assess the dermatologic features and associated psychological disorder.²

Psychogenic parasitosis

Also known as delusional parasitosis, psychogenic parasitosis is a psychodermatologic disorder in which patients believe they are infested with parasites. Patients with this disorder report seeing or feeling parasites on their skin, and they damage their skin in an attempt to remove them. Patients create excoriations and ulcers on easily reached areas, usually the ears, eyes, nose, and extremities. They often present with what is termed the “matchbox sign,” in which they bring containers filled with “small bits of excoriated skin, dried blood, debris, or insect parts as proof of infestation.”²

Women over the age of 50 years are more often affected with psychogenic parasitosis, and the disorder is associated with anxiety, depression, and hypochondriasis.

Factitial dermatitis

Factitial dermatitis, also known as dermatitis artefacta, is a psychodermatologic disorder in which patients damage their skin but deny their self-involvement. This disorder encompasses a wide range of lesions, including blisters, cuts, ulcers, and burns. Patients often are unable to describe how the lesions evolved. Lesions exhibit bizarre patterns not characteristic of any disease.

Young adults and adolescents are more commonly affected, and it is 4 times more common among women than men. Psychological disorders involved with factitial dermatitis include personality disorders and posttraumatic stress disorder.²

Neurotic excoriations

Neurotic excoriations, sometimes referred to as neurodermatitis, are a result of a psychodermatologic disorder in which patients inflict excoriations and ulcers on their skin and admit to their involvement.³ The condition is characterized by excoriations similar in size and shape that are localized on areas easily reached by the patient, such as the arms, legs, and upper back.

The lesions may present in various stages, varying from dugout ulcers to ulcers covered with crusts and surrounded by erythema to areas receding into depressed scars. These lesions are a result of repetitive scratching and digging by the patient, usually without an underlying physical pathology but sometimes initiated by pruritus.^1,2

Studies show the condition primarily affects women, with a mean onset between the ages of 30 to 45 years.¹ Common psychiatric problems associated with neurotic excoriations include significant social stress, depression, anxiety, and obsessive-compulsive disorder.

Diagnosis: Neurotic excoriations due to depression and stress

The patient’s history and physical examination suggests a diagnosis of neurotic excoriations due to the characteristics of the excoriations and ulcers on her arms and leg, her admission of their self-inflicted nature, and the associated depression and psychosocial stress.

Laboratory tests

Although there are no available laboratory tests to confirm a positive finding of neurotic excoriations, tests could be performed to disprove any systemic causes of pruritus and the resulting dermatological damage.⁵ These tests include complete blood count with differential, chemistry profile, determination of thyroid-stimulating hormone levels, fasting plasma glucose level, and skin biopsies.^1,

Laboratory tests for systemic causes of pruritus should be based on the patient’s physical and history, avoiding a broad approach.

Treatment: address the skin and the psyche

The treatment of neurotic excoriations requires a dual approach, addressing both the dermatological problems and the underlying psychological disorder.³ Supportive dermatologic care is necessary to avoid secondary complications and to ensure that the patient feels supported.³

Dermatologic care

Topical steroids can be helpful to decrease the pruritus and inflammation. The steroid strength should be chosen based upon the severity of the lesions and the thickness of the skin. Steroid ointments are preferred to creams when there are skin ulcers and deep excoriations.

If there is significant crusting or exudate, there is probably a secondary bacterial infection and oral antibiotics are indicated ( Figure 3). A first-generation cephalosporin or dicloxacillin should provide adequate coverage against the most likely organisms, Staphylococcus aureus and Streptococcus pyogenes.

FIGURE 3
Detail of lesions on the arm

Psychological care

The selective serotonin reuptake inhibitors produce a strong antipruritic response in patients with neurotic excoriations. Doxepin is a tricyclic antidepressant with one of the most powerful antihistamines for pruritus. When prescribing doxepin, limit the amount of medication dispensed at one time to minimize the risk of suicide.³ The patient should trim the fingernails to reduce the amount of damage caused by scratching and digging.

Treating the underlying psychological disorder requires supportive and empathic counseling. It may be necessary to collaborate with other mental health specialists. Management options include psychotropic medication, stress management courses, and referral to a psychiatrist. Patients with psychodermatologic disorders frequently resist referral to mental health professionals.³ If this is the case, family physicians are well positioned to help patients with psychological problems.

Alternative courses of treatment include hypnosis to disrupt the itch-scratch cycle, and acupuncture and supportive therapy to reduce underlying stressors.¹

Patient’s outcome

By showing support and concern for the patient’s health during the visit, the physician strengthened the relationship with the patient, who felt comfortable disclosing many of her concerns and troubles. She received prescriptions for cephalexin 500 mg orally 3 times daily, 0.1% triamcinolone ointment to be applied twice daily, and doxepin 25 mg once nightly. She also received a referral for counseling.

At first she refused to trim her fingernails, but as she began to see that her ulcers were self-inflicted, she reconsidered and agreed. In fact, she asked to borrow our nail clippers before leaving the office.

Corresponding author
Richard P. Usatine, MD, University of Texas Health Sceicnes Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900.

Note: A handout developed by the American Academy of Family Physicians for patients with neurotic excoriations is available to print or photocopy at the following website: www.aafp.org/afp/20011215/neurph.html.

A 55-year-old woman came to the clinic complaining of severe itching on her arms and legs. Although she itched throughout the day, it became intolerable at night, disrupting her sleep. She would sometimes scratch her arms and legs until exhaustion but could find no relief. Being outside in warm and sunny weather aggravated the problem. She had used moisturizers, emollients, and topical corticosteroids, but they only alleviated the itching temporarily. The itching began 10 months earlier, just after she finalized the divorce from her husband of 20 years.

Examination of the skin revealed numerous excoriations with ulcerations and xerosis on the arms and left leg (Figure 1 and 2). The excoriations were located extensively from the dorsum of her left foot to above the knee and bilaterally from the wrist to above the elbow. They also showed signs of infection. The patient admitted they were self-inflicted. The patient’s right leg had been amputated 5 years before after a car accident, and she wore a prosthetic leg. Examination of other areas showed nothing remarkable.

The patient readily admitted to a great deal of psychological distress. She described feeling depressed since her divorce. She has had difficulty securing a full-time job and has high anxiety about being able to pay her rent and bills.

FIGURE 1
Excoriations on the arms…

FIGURE 2
…and the left leg

WHAT IS THE DIAGNOSIS?
WHAT IS THE TREATMENT STRATEGY?

DIFFERENTIAL DIAGNOSIS: PSYCHODERMATOLOGIC DISORDER

The patient’s history and physical examination points to a psychodermatologic disorder. Psycho-dermatologic disorders are conditions involving an interaction between the mind and skin and are classified as:³

• Psychophysiologic disorders:skin disorders worsened by emotional stress
• Primary psychiatric disorders:usually caused by psychological conditions with self-induced skin damage
• Secondary psychiatric disorders: psychological problems developed as a consequence of a disfiguring skin disorder, which negatively effects self-esteem and body image.³

This patient’s excoriations and ulcers are due to self-mutilation. The differential diagnosis includes psychogenic parasitosis, factitial dermatitis, and neurotic excoriations. These 3 skin conditions are primary psychiatric symptoms, and proper diagnosis revolves around being able to assess the dermatologic features and associated psychological disorder.²

Psychogenic parasitosis

Also known as delusional parasitosis, psychogenic parasitosis is a psychodermatologic disorder in which patients believe they are infested with parasites. Patients with this disorder report seeing or feeling parasites on their skin, and they damage their skin in an attempt to remove them. Patients create excoriations and ulcers on easily reached areas, usually the ears, eyes, nose, and extremities. They often present with what is termed the “matchbox sign,” in which they bring containers filled with “small bits of excoriated skin, dried blood, debris, or insect parts as proof of infestation.”²

Women over the age of 50 years are more often affected with psychogenic parasitosis, and the disorder is associated with anxiety, depression, and hypochondriasis.

Factitial dermatitis

Factitial dermatitis, also known as dermatitis artefacta, is a psychodermatologic disorder in which patients damage their skin but deny their self-involvement. This disorder encompasses a wide range of lesions, including blisters, cuts, ulcers, and burns. Patients often are unable to describe how the lesions evolved. Lesions exhibit bizarre patterns not characteristic of any disease.

Young adults and adolescents are more commonly affected, and it is 4 times more common among women than men. Psychological disorders involved with factitial dermatitis include personality disorders and posttraumatic stress disorder.²

Neurotic excoriations

Neurotic excoriations, sometimes referred to as neurodermatitis, are a result of a psychodermatologic disorder in which patients inflict excoriations and ulcers on their skin and admit to their involvement.³ The condition is characterized by excoriations similar in size and shape that are localized on areas easily reached by the patient, such as the arms, legs, and upper back.

The lesions may present in various stages, varying from dugout ulcers to ulcers covered with crusts and surrounded by erythema to areas receding into depressed scars. These lesions are a result of repetitive scratching and digging by the patient, usually without an underlying physical pathology but sometimes initiated by pruritus.^1,2

Studies show the condition primarily affects women, with a mean onset between the ages of 30 to 45 years.¹ Common psychiatric problems associated with neurotic excoriations include significant social stress, depression, anxiety, and obsessive-compulsive disorder.

Diagnosis: Neurotic excoriations due to depression and stress

The patient’s history and physical examination suggests a diagnosis of neurotic excoriations due to the characteristics of the excoriations and ulcers on her arms and leg, her admission of their self-inflicted nature, and the associated depression and psychosocial stress.

Laboratory tests

Although there are no available laboratory tests to confirm a positive finding of neurotic excoriations, tests could be performed to disprove any systemic causes of pruritus and the resulting dermatological damage.⁵ These tests include complete blood count with differential, chemistry profile, determination of thyroid-stimulating hormone levels, fasting plasma glucose level, and skin biopsies.^1,

Laboratory tests for systemic causes of pruritus should be based on the patient’s physical and history, avoiding a broad approach.

Treatment: address the skin and the psyche

The treatment of neurotic excoriations requires a dual approach, addressing both the dermatological problems and the underlying psychological disorder.³ Supportive dermatologic care is necessary to avoid secondary complications and to ensure that the patient feels supported.³

Dermatologic care

Topical steroids can be helpful to decrease the pruritus and inflammation. The steroid strength should be chosen based upon the severity of the lesions and the thickness of the skin. Steroid ointments are preferred to creams when there are skin ulcers and deep excoriations.

If there is significant crusting or exudate, there is probably a secondary bacterial infection and oral antibiotics are indicated ( Figure 3). A first-generation cephalosporin or dicloxacillin should provide adequate coverage against the most likely organisms, Staphylococcus aureus and Streptococcus pyogenes.

FIGURE 3
Detail of lesions on the arm

Psychological care

The selective serotonin reuptake inhibitors produce a strong antipruritic response in patients with neurotic excoriations. Doxepin is a tricyclic antidepressant with one of the most powerful antihistamines for pruritus. When prescribing doxepin, limit the amount of medication dispensed at one time to minimize the risk of suicide.³ The patient should trim the fingernails to reduce the amount of damage caused by scratching and digging.

Treating the underlying psychological disorder requires supportive and empathic counseling. It may be necessary to collaborate with other mental health specialists. Management options include psychotropic medication, stress management courses, and referral to a psychiatrist. Patients with psychodermatologic disorders frequently resist referral to mental health professionals.³ If this is the case, family physicians are well positioned to help patients with psychological problems.

Alternative courses of treatment include hypnosis to disrupt the itch-scratch cycle, and acupuncture and supportive therapy to reduce underlying stressors.¹

Patient’s outcome

By showing support and concern for the patient’s health during the visit, the physician strengthened the relationship with the patient, who felt comfortable disclosing many of her concerns and troubles. She received prescriptions for cephalexin 500 mg orally 3 times daily, 0.1% triamcinolone ointment to be applied twice daily, and doxepin 25 mg once nightly. She also received a referral for counseling.

At first she refused to trim her fingernails, but as she began to see that her ulcers were self-inflicted, she reconsidered and agreed. In fact, she asked to borrow our nail clippers before leaving the office.

Corresponding author
Richard P. Usatine, MD, University of Texas Health Sceicnes Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900.

Note: A handout developed by the American Academy of Family Physicians for patients with neurotic excoriations is available to print or photocopy at the following website: www.aafp.org/afp/20011215/neurph.html.

A 67-year-old woman came to the office with pain in her hands. She had just arrived from Panama to live with her son. She has had this pain for decades: she refers to it as artritisin Spanish but does not know what type of arthritis. Aspirin had helped in the past, but lately she had not been getting enough relief from it. Her hands feel stiff in the morning for at least 1 hour, which interferes with cooking and sewing.

Her hands showed signs of joint swelling and deformities (Figure 1). Her swollen joints felt warm. She also had knee pain.

FIGURE 1
Painful,swollen hands

What type of arthritis does she have?
Are any diagnostic tests necessary?
What are the best treatments available?

That same day, another patient was seen with painful hands (Figure 2). What type of arthritis does she have, and how does it differ from the condition of the patient in Figure 1 ?

FIGURE 2
Another patient with hand pain

The obvious ulnar deviation of her fingers and the swelling of the metacarpophalangeal (MCP) joints (Figure 1) are strongly indicative of rheumatoid arthritis. The patient in Figure 2 has swelling and deformities in her proximal inter-phalangeal (PIP) and distal interphalangeal (DIP) joints, indicating that she most likely has osteoarthritis. The swelling of the PIP joints is called Bouchard’s nodes; the swelling of the DIP joints is called Heberden’s nodes.

Differential diagnosis: types of arthritis

The first decision point in diagnosing chronic (>6 weeks) polyarticular joint pain is distinguishing between inflammatory and noninflammatory arthritis. Key features of inflammatory arthritis are stiffness in the morning or after inactivity, and visible joint swelling. The differential diagnosis of inflammatory arthritis includes rheumatoid arthritis, psoriatic arthritis, seronegative spondyloarthropathies, and systemic lupus erythematosus (SLE).

After the age of 50, maturity-onset seronegative synovitis syndrome and crystal-induced synovitis should also be considered.¹ Although osteoarthritis is considered noninflammatory, inflammation of the joint tissue occurs occasionally due to the joint’s degenerative loss of cartilage and bony overgrowth.

It is critical to identify rheumatoid arthritis early, as prompt intervention can delay disease progression, and reduce the substantial morbidity and mortality of rheumatoid arthritis.² A diagnosis of rheumatoid arthritis requires 4 of the following: morning stiffness; arthritis in 3 or more joints; arthritis in the wrist, MCP joints, or PIP joints; symmetric arthritis; rheumatoid nodules; positive rheumatoid factor; radiographic changes.¹ This patient has the morning stiffness, arthritis in more than 3 joints, symmetric arthritis, and rheumatoid nodules on her feet (Figure 3).

The most common noninflammatory arthritis is osteoarthritis, which affects 21 million Americans.² The weight-bearing joints are usually affected, and damage may occur because of trauma or repetitive impact. When the hands are involved, the DIP and PIP joints are more likely to be involved than the MCP joints.

FIGURE 3
Rheumatoid nodules on feet

Diagnostic tests can differentiate between causes

Laboratory tests can help differentiate between conditions causing inflammatory arthritis. Rheumatoid factor is positive in 70% of patients with rheumatoid arthritis, and the antinuclear antibody is invariably positive for patients with SLE. Maturity-onset seronegative synovitis has negative rheumatoid factor and antinuclear antibody tests with marked elevation in erythrocyte sedimentation rate. Polyarticular gout may have increased serum uric acid, and is best diagnosed by demonstrating crystals in the joint fluid.

Laboratory tests are not helpful in diagnosing psoriatic arthritis, seronegative spondylo-arthropathies, and osteoarthritis with inflammation.¹ Radiographic studies may show joint erosions in patients with active rheumatoid arthritis for more than a year, and typical osteophytes and joint-space narrowing in osteoarthritis.¹

Radiographs are not necessary for the diagnosis of this patient but may help management, especially if hand surgery is going to be considered. In this case, the radiographs showed joint erosions and unequivocal juxta-auricular osteopenia.

Management: decrease pain, optimize mobility

The management of osteoarthritis and rheumatoid arthritis is different. However, in all types of arthritis the goals of therapy are to decrease pain, optimize mobility, and maximize quality of life. In rheumatoid arthritis, another goal is to slow the progression of the disease with disease-modifying antirheumatic drugs.

Osteoarthritis. First-line therapy for osteo arthritis includes exercise, weight loss (if indicated), and acetaminophen in scheduled doses up to 1000 mg 4 times a day. A recent Cochrane Review concluded that acetaminophen is clearly superior to placebo, but slightly less efficacious than nonsteroidal anti-inflammatory drugs (NSAIDs) for pain relief in osteoarthritis (level of evidence [LOE]: 1a). Acetaminophen and NSAIDs were equivalent in improving function. This evidence supports the use of acetaminophen first, reserving NSAIDs for those who do not respond.³ Adding NSAIDs may improve pain relief, but carries an increased risk of gastrointestinal ulcerations or bleeding.

A cyclo-oxygenase-2 (COX-2) inhibitor may be preferred to NSAIDs for patients at high risk for gastrointestinal complications. Other treatments include topical analgesics, glucosamine, and chondroitin. Large clinical trials for glucosamine and chondroitin are ongoing.

Rheumatoid arthritis. In rheumatoid arthritis, the recommendation from the American College of Rheumatology is for early, aggressive intervention with disease-modifying antirheumatic drugs, often within a few months of the onset of the disease.⁴ Unfortunately, the patient in Figure 1 has had rheumatoid arthritis for decades. Patients with rheumatoid arthritis also benefit from exercise and physical therapy.

Other treatments include low-dose corticosteroids and NSAIDs or COX-2 inhibitors. COX-2 inhibitors have similar efficacy to NSAIDs, with a lower risk of gastrointestinal complications (LOE: 1a).^5,6 COX-2 inhibitors should be considered in place of NSAIDs for patients with rheumatoid arthritis, as these patients are almost twice as likely to suffer from serious gastrointestinal complications as the general population.² COX-2 inhibitors, however, are much more expensive than NSAIDs, which limits their use.

Patient’s outcome

The patient was started on an anti-inflammatory medication and referred to a rheumatologist for consideration of a disease-modifying antirheumatic drug.

Acknowledgments

The authors would like to acknowledge Michael Fischbach, MD, in the Rheumatology Department of the University of Texas Health Science Center for his contribution to this article.

Correspondence
Richard P. Usatine, Editor, Photo Rounds, University of Texas HealthSciences Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd CurlDrive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 67-year-old woman came to the office with pain in her hands. She had just arrived from Panama to live with her son. She has had this pain for decades: she refers to it as artritisin Spanish but does not know what type of arthritis. Aspirin had helped in the past, but lately she had not been getting enough relief from it. Her hands feel stiff in the morning for at least 1 hour, which interferes with cooking and sewing.

Her hands showed signs of joint swelling and deformities (Figure 1). Her swollen joints felt warm. She also had knee pain.

FIGURE 1
Painful,swollen hands

What type of arthritis does she have?
Are any diagnostic tests necessary?
What are the best treatments available?

That same day, another patient was seen with painful hands (Figure 2). What type of arthritis does she have, and how does it differ from the condition of the patient in Figure 1 ?

FIGURE 2
Another patient with hand pain

The obvious ulnar deviation of her fingers and the swelling of the metacarpophalangeal (MCP) joints (Figure 1) are strongly indicative of rheumatoid arthritis. The patient in Figure 2 has swelling and deformities in her proximal inter-phalangeal (PIP) and distal interphalangeal (DIP) joints, indicating that she most likely has osteoarthritis. The swelling of the PIP joints is called Bouchard’s nodes; the swelling of the DIP joints is called Heberden’s nodes.

Differential diagnosis: types of arthritis

The first decision point in diagnosing chronic (>6 weeks) polyarticular joint pain is distinguishing between inflammatory and noninflammatory arthritis. Key features of inflammatory arthritis are stiffness in the morning or after inactivity, and visible joint swelling. The differential diagnosis of inflammatory arthritis includes rheumatoid arthritis, psoriatic arthritis, seronegative spondyloarthropathies, and systemic lupus erythematosus (SLE).

After the age of 50, maturity-onset seronegative synovitis syndrome and crystal-induced synovitis should also be considered.¹ Although osteoarthritis is considered noninflammatory, inflammation of the joint tissue occurs occasionally due to the joint’s degenerative loss of cartilage and bony overgrowth.

It is critical to identify rheumatoid arthritis early, as prompt intervention can delay disease progression, and reduce the substantial morbidity and mortality of rheumatoid arthritis.² A diagnosis of rheumatoid arthritis requires 4 of the following: morning stiffness; arthritis in 3 or more joints; arthritis in the wrist, MCP joints, or PIP joints; symmetric arthritis; rheumatoid nodules; positive rheumatoid factor; radiographic changes.¹ This patient has the morning stiffness, arthritis in more than 3 joints, symmetric arthritis, and rheumatoid nodules on her feet (Figure 3).

The most common noninflammatory arthritis is osteoarthritis, which affects 21 million Americans.² The weight-bearing joints are usually affected, and damage may occur because of trauma or repetitive impact. When the hands are involved, the DIP and PIP joints are more likely to be involved than the MCP joints.

FIGURE 3
Rheumatoid nodules on feet

Diagnostic tests can differentiate between causes

Laboratory tests can help differentiate between conditions causing inflammatory arthritis. Rheumatoid factor is positive in 70% of patients with rheumatoid arthritis, and the antinuclear antibody is invariably positive for patients with SLE. Maturity-onset seronegative synovitis has negative rheumatoid factor and antinuclear antibody tests with marked elevation in erythrocyte sedimentation rate. Polyarticular gout may have increased serum uric acid, and is best diagnosed by demonstrating crystals in the joint fluid.

Laboratory tests are not helpful in diagnosing psoriatic arthritis, seronegative spondylo-arthropathies, and osteoarthritis with inflammation.¹ Radiographic studies may show joint erosions in patients with active rheumatoid arthritis for more than a year, and typical osteophytes and joint-space narrowing in osteoarthritis.¹

Radiographs are not necessary for the diagnosis of this patient but may help management, especially if hand surgery is going to be considered. In this case, the radiographs showed joint erosions and unequivocal juxta-auricular osteopenia.

Management: decrease pain, optimize mobility

The management of osteoarthritis and rheumatoid arthritis is different. However, in all types of arthritis the goals of therapy are to decrease pain, optimize mobility, and maximize quality of life. In rheumatoid arthritis, another goal is to slow the progression of the disease with disease-modifying antirheumatic drugs.

Osteoarthritis. First-line therapy for osteo arthritis includes exercise, weight loss (if indicated), and acetaminophen in scheduled doses up to 1000 mg 4 times a day. A recent Cochrane Review concluded that acetaminophen is clearly superior to placebo, but slightly less efficacious than nonsteroidal anti-inflammatory drugs (NSAIDs) for pain relief in osteoarthritis (level of evidence [LOE]: 1a). Acetaminophen and NSAIDs were equivalent in improving function. This evidence supports the use of acetaminophen first, reserving NSAIDs for those who do not respond.³ Adding NSAIDs may improve pain relief, but carries an increased risk of gastrointestinal ulcerations or bleeding.

A cyclo-oxygenase-2 (COX-2) inhibitor may be preferred to NSAIDs for patients at high risk for gastrointestinal complications. Other treatments include topical analgesics, glucosamine, and chondroitin. Large clinical trials for glucosamine and chondroitin are ongoing.

Rheumatoid arthritis. In rheumatoid arthritis, the recommendation from the American College of Rheumatology is for early, aggressive intervention with disease-modifying antirheumatic drugs, often within a few months of the onset of the disease.⁴ Unfortunately, the patient in Figure 1 has had rheumatoid arthritis for decades. Patients with rheumatoid arthritis also benefit from exercise and physical therapy.

Other treatments include low-dose corticosteroids and NSAIDs or COX-2 inhibitors. COX-2 inhibitors have similar efficacy to NSAIDs, with a lower risk of gastrointestinal complications (LOE: 1a).^5,6 COX-2 inhibitors should be considered in place of NSAIDs for patients with rheumatoid arthritis, as these patients are almost twice as likely to suffer from serious gastrointestinal complications as the general population.² COX-2 inhibitors, however, are much more expensive than NSAIDs, which limits their use.

Patient’s outcome

The patient was started on an anti-inflammatory medication and referred to a rheumatologist for consideration of a disease-modifying antirheumatic drug.

Acknowledgments

The authors would like to acknowledge Michael Fischbach, MD, in the Rheumatology Department of the University of Texas Health Science Center for his contribution to this article.

Correspondence
Richard P. Usatine, Editor, Photo Rounds, University of Texas HealthSciences Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd CurlDrive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 67-year-old woman came to the office with pain in her hands. She had just arrived from Panama to live with her son. She has had this pain for decades: she refers to it as artritisin Spanish but does not know what type of arthritis. Aspirin had helped in the past, but lately she had not been getting enough relief from it. Her hands feel stiff in the morning for at least 1 hour, which interferes with cooking and sewing.

Her hands showed signs of joint swelling and deformities (Figure 1). Her swollen joints felt warm. She also had knee pain.

FIGURE 1
Painful,swollen hands

What type of arthritis does she have?
Are any diagnostic tests necessary?
What are the best treatments available?

That same day, another patient was seen with painful hands (Figure 2). What type of arthritis does she have, and how does it differ from the condition of the patient in Figure 1 ?

FIGURE 2
Another patient with hand pain

The obvious ulnar deviation of her fingers and the swelling of the metacarpophalangeal (MCP) joints (Figure 1) are strongly indicative of rheumatoid arthritis. The patient in Figure 2 has swelling and deformities in her proximal inter-phalangeal (PIP) and distal interphalangeal (DIP) joints, indicating that she most likely has osteoarthritis. The swelling of the PIP joints is called Bouchard’s nodes; the swelling of the DIP joints is called Heberden’s nodes.

Differential diagnosis: types of arthritis

The first decision point in diagnosing chronic (>6 weeks) polyarticular joint pain is distinguishing between inflammatory and noninflammatory arthritis. Key features of inflammatory arthritis are stiffness in the morning or after inactivity, and visible joint swelling. The differential diagnosis of inflammatory arthritis includes rheumatoid arthritis, psoriatic arthritis, seronegative spondyloarthropathies, and systemic lupus erythematosus (SLE).

After the age of 50, maturity-onset seronegative synovitis syndrome and crystal-induced synovitis should also be considered.¹ Although osteoarthritis is considered noninflammatory, inflammation of the joint tissue occurs occasionally due to the joint’s degenerative loss of cartilage and bony overgrowth.

It is critical to identify rheumatoid arthritis early, as prompt intervention can delay disease progression, and reduce the substantial morbidity and mortality of rheumatoid arthritis.² A diagnosis of rheumatoid arthritis requires 4 of the following: morning stiffness; arthritis in 3 or more joints; arthritis in the wrist, MCP joints, or PIP joints; symmetric arthritis; rheumatoid nodules; positive rheumatoid factor; radiographic changes.¹ This patient has the morning stiffness, arthritis in more than 3 joints, symmetric arthritis, and rheumatoid nodules on her feet (Figure 3).

The most common noninflammatory arthritis is osteoarthritis, which affects 21 million Americans.² The weight-bearing joints are usually affected, and damage may occur because of trauma or repetitive impact. When the hands are involved, the DIP and PIP joints are more likely to be involved than the MCP joints.

FIGURE 3
Rheumatoid nodules on feet

Diagnostic tests can differentiate between causes

Laboratory tests can help differentiate between conditions causing inflammatory arthritis. Rheumatoid factor is positive in 70% of patients with rheumatoid arthritis, and the antinuclear antibody is invariably positive for patients with SLE. Maturity-onset seronegative synovitis has negative rheumatoid factor and antinuclear antibody tests with marked elevation in erythrocyte sedimentation rate. Polyarticular gout may have increased serum uric acid, and is best diagnosed by demonstrating crystals in the joint fluid.

Laboratory tests are not helpful in diagnosing psoriatic arthritis, seronegative spondylo-arthropathies, and osteoarthritis with inflammation.¹ Radiographic studies may show joint erosions in patients with active rheumatoid arthritis for more than a year, and typical osteophytes and joint-space narrowing in osteoarthritis.¹

Radiographs are not necessary for the diagnosis of this patient but may help management, especially if hand surgery is going to be considered. In this case, the radiographs showed joint erosions and unequivocal juxta-auricular osteopenia.

Management: decrease pain, optimize mobility

The management of osteoarthritis and rheumatoid arthritis is different. However, in all types of arthritis the goals of therapy are to decrease pain, optimize mobility, and maximize quality of life. In rheumatoid arthritis, another goal is to slow the progression of the disease with disease-modifying antirheumatic drugs.

Osteoarthritis. First-line therapy for osteo arthritis includes exercise, weight loss (if indicated), and acetaminophen in scheduled doses up to 1000 mg 4 times a day. A recent Cochrane Review concluded that acetaminophen is clearly superior to placebo, but slightly less efficacious than nonsteroidal anti-inflammatory drugs (NSAIDs) for pain relief in osteoarthritis (level of evidence [LOE]: 1a). Acetaminophen and NSAIDs were equivalent in improving function. This evidence supports the use of acetaminophen first, reserving NSAIDs for those who do not respond.³ Adding NSAIDs may improve pain relief, but carries an increased risk of gastrointestinal ulcerations or bleeding.

A cyclo-oxygenase-2 (COX-2) inhibitor may be preferred to NSAIDs for patients at high risk for gastrointestinal complications. Other treatments include topical analgesics, glucosamine, and chondroitin. Large clinical trials for glucosamine and chondroitin are ongoing.

Rheumatoid arthritis. In rheumatoid arthritis, the recommendation from the American College of Rheumatology is for early, aggressive intervention with disease-modifying antirheumatic drugs, often within a few months of the onset of the disease.⁴ Unfortunately, the patient in Figure 1 has had rheumatoid arthritis for decades. Patients with rheumatoid arthritis also benefit from exercise and physical therapy.

Other treatments include low-dose corticosteroids and NSAIDs or COX-2 inhibitors. COX-2 inhibitors have similar efficacy to NSAIDs, with a lower risk of gastrointestinal complications (LOE: 1a).^5,6 COX-2 inhibitors should be considered in place of NSAIDs for patients with rheumatoid arthritis, as these patients are almost twice as likely to suffer from serious gastrointestinal complications as the general population.² COX-2 inhibitors, however, are much more expensive than NSAIDs, which limits their use.

Patient’s outcome

The patient was started on an anti-inflammatory medication and referred to a rheumatologist for consideration of a disease-modifying antirheumatic drug.

Acknowledgments

The authors would like to acknowledge Michael Fischbach, MD, in the Rheumatology Department of the University of Texas Health Science Center for his contribution to this article.

Correspondence
Richard P. Usatine, Editor, Photo Rounds, University of Texas HealthSciences Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd CurlDrive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu

A 32-year-old woman came to the rescue mission clinic with her 2 sons because she had red eyes and a runny nose. Her sons both had symptoms highly suggestive of viral upper respiratory infection. They lived in a homeless shelter.

The patient stated she did not use contact lenses or have any eye trauma, itching, photophobia, loss or change of vision, eye pain, eye discharge, or previous episodes of pinkeye. She had no other medical problems or history of allergies.

On physical exam, her vital signs were normal. She had conjunctival injection, without purulent discharge or limbal blush (Figures 1 and 2). Eyelids were mildly erythematous with no cobble-stoning. Pupils were equal, round, and reactive to light. The anterior chamber by flashlight exam from the side did not show a narrow angle. Visual acuity was normal by Snellen exam. She had clear nasal discharge and bilateral preauricular lymphadenopathy.

In addition, she had a brown macule under the left iris on the conjunctiva. The patient said this had been present since childhood and it had not changed.

FIGURE 1
Red eyes

FIGURE 2
Brown macule

What is the differential iagnosis?
What about the brown macule?
Are any diagnostic tests Necessary for either condition?

Differential diagnosis

The differential diagnosis of a red eye includes:

• Conjunctivitis
• Uveitis
• Acute glaucoma
• Corneal disease or foreign body trauma
• Scleritis and episcleritis.¹

For this patient, conjunctivitis is the most likely diagnosis. The absence of eye pain or loss of vision makes uveitis, acute glaucoma, or corneal disease (including foreign-body trauma) less likely. The round shape of the pupil and the absence of the limbal blush also make uveitis less likely. The pattern of injection does not match the wedge-shaped inflammation of episcleritis or the depth of scleritis.

Diagnosis: viral conjunctivitis

This patient has viral conjunctivitis. Conjunctivitis can be infectious, allergic, chemical/irritative, or autoimmune in origin. The most common infectious agents are viral—specifically, the adenoviruses. Other infectious agents include bacteria (Staphylococcus aureus, Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria gonorrhoeae), chlamydia, and herpes simplex virus.

Both infectious and allergic conjunctivitis are common. In this patient, the presence of nasal discharge, preauricular lymphadenopathy, and the lack of pruritus make viral infection more likely than allergic. Conjunctivitis that is bilateral without purulent discharge is more likely to be viral than bacterial.

What about the brown macule?

This patient also had a brown macule on her conjunctiva. The differential diagnosis of pigmented areas on the conjunctiva includes nevus, racial melanosis, primary acquired melanosis, secondary pigmentary deposition, and ocular melanoma.² These conditions (besides ocular melanoma) are benign.

Although ocular melanoma is the most common primary intraocular malignancy in adults, and the second most likely location for primary melanoma after the skin, it is still exceedingly rare. In the Causasian population, it has an average annual incidence of 6 cases per million, with approximately 1200 cases diagnosed each year. Ocular melanoma occurs in the uvea much more commonly than in the conjunctiva, at a ratio of 35:1. Conjunctival melanomas have a propensity for regional spread to the lymph nodes analogous to cutaneous disease, with 10-year survival rates of more than 80%.³

In this case, the patient had this dark spot since childhood and had noted no growth or change. It was consistent with a conjunctival nevus and did not need biopsy.

Diagnostic tests: only for cases that do not respond

When viral conjunctivitis is suspected, no laboratory tests are routinely recommended. Bacterial and viral cultures may be helpful to establish the diagnosis in cases that do not resolve or when patients have recurrent episodes. Infections that do not respond to empiric treatment should be cultured for the suspected organisms (bacteria, chlamydia, or herpes simplex). When chlamydial conjunctivitis is suspected, the diagnosis should be confirmed by means of an immunodiagnostic test (direct fluorescent antibody [DFA]) or culture (level of evidence [LOE]=1a).⁴

A fluorescein exam is helpful in cases with a question of corneal involvement from foreign-body trauma, herpes simplex, or epidemic kerato-conjunctivitis. Herpes simplex infections have a dendritic pattern of ulceration, and epidemic keratoconjunctivitis infections cause multiple small areas of increased fluorescein uptake.

Management: conjunctivitis usually self-limiting

Typical viral conjunctivitis caused by the adenoviruses or other common viruses (not herpes) does not require medication. Warm compresses may be recommended to reduce discomfort. Infectious conjunctivitis caused by bacteria are also usually self-limiting; however, a recent meta-analysis indicates that treatment with antibiotics can shorten the clinical duration (LOE=1a).⁵

Appropriate medications for bacterial conjunctivitis include 0.3% tobramycin or gentamycin, 10% sodium sulfacetamide, or erythromycin ophthalmic ointment. If herpetic keratoconjuntivitis is suspected, prompt ophthalmologic referral is indicated.¹

Patient’s treatment and outcome

This patient was managed with reassurance and symptomatic treatment of her viral respiratory illness. Her red eyes and upper respiratory infection both resolved spontaneously within 1 week.

As her nevus had not changed in many years, she was instructed to continue to watch the nevus and report any changes to a physician for evaluation. If the lesion changed in the future, she should be referred to an ophthalmologist.

Correspondence
Richard P. Usatine, Editor, Photo Rounds, University of Texas Health Sciences Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu.

A 32-year-old woman came to the rescue mission clinic with her 2 sons because she had red eyes and a runny nose. Her sons both had symptoms highly suggestive of viral upper respiratory infection. They lived in a homeless shelter.

The patient stated she did not use contact lenses or have any eye trauma, itching, photophobia, loss or change of vision, eye pain, eye discharge, or previous episodes of pinkeye. She had no other medical problems or history of allergies.

On physical exam, her vital signs were normal. She had conjunctival injection, without purulent discharge or limbal blush (Figures 1 and 2). Eyelids were mildly erythematous with no cobble-stoning. Pupils were equal, round, and reactive to light. The anterior chamber by flashlight exam from the side did not show a narrow angle. Visual acuity was normal by Snellen exam. She had clear nasal discharge and bilateral preauricular lymphadenopathy.

In addition, she had a brown macule under the left iris on the conjunctiva. The patient said this had been present since childhood and it had not changed.

FIGURE 1
Red eyes

FIGURE 2
Brown macule

What is the differential iagnosis?
What about the brown macule?
Are any diagnostic tests Necessary for either condition?

Differential diagnosis

The differential diagnosis of a red eye includes:

• Conjunctivitis
• Uveitis
• Acute glaucoma
• Corneal disease or foreign body trauma
• Scleritis and episcleritis.¹

For this patient, conjunctivitis is the most likely diagnosis. The absence of eye pain or loss of vision makes uveitis, acute glaucoma, or corneal disease (including foreign-body trauma) less likely. The round shape of the pupil and the absence of the limbal blush also make uveitis less likely. The pattern of injection does not match the wedge-shaped inflammation of episcleritis or the depth of scleritis.

Diagnosis: viral conjunctivitis

This patient has viral conjunctivitis. Conjunctivitis can be infectious, allergic, chemical/irritative, or autoimmune in origin. The most common infectious agents are viral—specifically, the adenoviruses. Other infectious agents include bacteria (Staphylococcus aureus, Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria gonorrhoeae), chlamydia, and herpes simplex virus.

Both infectious and allergic conjunctivitis are common. In this patient, the presence of nasal discharge, preauricular lymphadenopathy, and the lack of pruritus make viral infection more likely than allergic. Conjunctivitis that is bilateral without purulent discharge is more likely to be viral than bacterial.

What about the brown macule?

This patient also had a brown macule on her conjunctiva. The differential diagnosis of pigmented areas on the conjunctiva includes nevus, racial melanosis, primary acquired melanosis, secondary pigmentary deposition, and ocular melanoma.² These conditions (besides ocular melanoma) are benign.

Although ocular melanoma is the most common primary intraocular malignancy in adults, and the second most likely location for primary melanoma after the skin, it is still exceedingly rare. In the Causasian population, it has an average annual incidence of 6 cases per million, with approximately 1200 cases diagnosed each year. Ocular melanoma occurs in the uvea much more commonly than in the conjunctiva, at a ratio of 35:1. Conjunctival melanomas have a propensity for regional spread to the lymph nodes analogous to cutaneous disease, with 10-year survival rates of more than 80%.³

In this case, the patient had this dark spot since childhood and had noted no growth or change. It was consistent with a conjunctival nevus and did not need biopsy.

Diagnostic tests: only for cases that do not respond

When viral conjunctivitis is suspected, no laboratory tests are routinely recommended. Bacterial and viral cultures may be helpful to establish the diagnosis in cases that do not resolve or when patients have recurrent episodes. Infections that do not respond to empiric treatment should be cultured for the suspected organisms (bacteria, chlamydia, or herpes simplex). When chlamydial conjunctivitis is suspected, the diagnosis should be confirmed by means of an immunodiagnostic test (direct fluorescent antibody [DFA]) or culture (level of evidence [LOE]=1a).⁴

A fluorescein exam is helpful in cases with a question of corneal involvement from foreign-body trauma, herpes simplex, or epidemic kerato-conjunctivitis. Herpes simplex infections have a dendritic pattern of ulceration, and epidemic keratoconjunctivitis infections cause multiple small areas of increased fluorescein uptake.

Management: conjunctivitis usually self-limiting

Typical viral conjunctivitis caused by the adenoviruses or other common viruses (not herpes) does not require medication. Warm compresses may be recommended to reduce discomfort. Infectious conjunctivitis caused by bacteria are also usually self-limiting; however, a recent meta-analysis indicates that treatment with antibiotics can shorten the clinical duration (LOE=1a).⁵

Appropriate medications for bacterial conjunctivitis include 0.3% tobramycin or gentamycin, 10% sodium sulfacetamide, or erythromycin ophthalmic ointment. If herpetic keratoconjuntivitis is suspected, prompt ophthalmologic referral is indicated.¹

Patient’s treatment and outcome

This patient was managed with reassurance and symptomatic treatment of her viral respiratory illness. Her red eyes and upper respiratory infection both resolved spontaneously within 1 week.

As her nevus had not changed in many years, she was instructed to continue to watch the nevus and report any changes to a physician for evaluation. If the lesion changed in the future, she should be referred to an ophthalmologist.

Correspondence
Richard P. Usatine, Editor, Photo Rounds, University of Texas Health Sciences Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu.

A 32-year-old woman came to the rescue mission clinic with her 2 sons because she had red eyes and a runny nose. Her sons both had symptoms highly suggestive of viral upper respiratory infection. They lived in a homeless shelter.

The patient stated she did not use contact lenses or have any eye trauma, itching, photophobia, loss or change of vision, eye pain, eye discharge, or previous episodes of pinkeye. She had no other medical problems or history of allergies.

On physical exam, her vital signs were normal. She had conjunctival injection, without purulent discharge or limbal blush (Figures 1 and 2). Eyelids were mildly erythematous with no cobble-stoning. Pupils were equal, round, and reactive to light. The anterior chamber by flashlight exam from the side did not show a narrow angle. Visual acuity was normal by Snellen exam. She had clear nasal discharge and bilateral preauricular lymphadenopathy.

In addition, she had a brown macule under the left iris on the conjunctiva. The patient said this had been present since childhood and it had not changed.

FIGURE 1
Red eyes

FIGURE 2
Brown macule

What is the differential iagnosis?
What about the brown macule?
Are any diagnostic tests Necessary for either condition?

Differential diagnosis

The differential diagnosis of a red eye includes:

• Conjunctivitis
• Uveitis
• Acute glaucoma
• Corneal disease or foreign body trauma
• Scleritis and episcleritis.¹

For this patient, conjunctivitis is the most likely diagnosis. The absence of eye pain or loss of vision makes uveitis, acute glaucoma, or corneal disease (including foreign-body trauma) less likely. The round shape of the pupil and the absence of the limbal blush also make uveitis less likely. The pattern of injection does not match the wedge-shaped inflammation of episcleritis or the depth of scleritis.

Diagnosis: viral conjunctivitis

This patient has viral conjunctivitis. Conjunctivitis can be infectious, allergic, chemical/irritative, or autoimmune in origin. The most common infectious agents are viral—specifically, the adenoviruses. Other infectious agents include bacteria (Staphylococcus aureus, Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria gonorrhoeae), chlamydia, and herpes simplex virus.

Both infectious and allergic conjunctivitis are common. In this patient, the presence of nasal discharge, preauricular lymphadenopathy, and the lack of pruritus make viral infection more likely than allergic. Conjunctivitis that is bilateral without purulent discharge is more likely to be viral than bacterial.

What about the brown macule?

This patient also had a brown macule on her conjunctiva. The differential diagnosis of pigmented areas on the conjunctiva includes nevus, racial melanosis, primary acquired melanosis, secondary pigmentary deposition, and ocular melanoma.² These conditions (besides ocular melanoma) are benign.

Although ocular melanoma is the most common primary intraocular malignancy in adults, and the second most likely location for primary melanoma after the skin, it is still exceedingly rare. In the Causasian population, it has an average annual incidence of 6 cases per million, with approximately 1200 cases diagnosed each year. Ocular melanoma occurs in the uvea much more commonly than in the conjunctiva, at a ratio of 35:1. Conjunctival melanomas have a propensity for regional spread to the lymph nodes analogous to cutaneous disease, with 10-year survival rates of more than 80%.³

In this case, the patient had this dark spot since childhood and had noted no growth or change. It was consistent with a conjunctival nevus and did not need biopsy.

Diagnostic tests: only for cases that do not respond

When viral conjunctivitis is suspected, no laboratory tests are routinely recommended. Bacterial and viral cultures may be helpful to establish the diagnosis in cases that do not resolve or when patients have recurrent episodes. Infections that do not respond to empiric treatment should be cultured for the suspected organisms (bacteria, chlamydia, or herpes simplex). When chlamydial conjunctivitis is suspected, the diagnosis should be confirmed by means of an immunodiagnostic test (direct fluorescent antibody [DFA]) or culture (level of evidence [LOE]=1a).⁴

A fluorescein exam is helpful in cases with a question of corneal involvement from foreign-body trauma, herpes simplex, or epidemic kerato-conjunctivitis. Herpes simplex infections have a dendritic pattern of ulceration, and epidemic keratoconjunctivitis infections cause multiple small areas of increased fluorescein uptake.

Management: conjunctivitis usually self-limiting

Typical viral conjunctivitis caused by the adenoviruses or other common viruses (not herpes) does not require medication. Warm compresses may be recommended to reduce discomfort. Infectious conjunctivitis caused by bacteria are also usually self-limiting; however, a recent meta-analysis indicates that treatment with antibiotics can shorten the clinical duration (LOE=1a).⁵

Appropriate medications for bacterial conjunctivitis include 0.3% tobramycin or gentamycin, 10% sodium sulfacetamide, or erythromycin ophthalmic ointment. If herpetic keratoconjuntivitis is suspected, prompt ophthalmologic referral is indicated.¹

Patient’s treatment and outcome

This patient was managed with reassurance and symptomatic treatment of her viral respiratory illness. Her red eyes and upper respiratory infection both resolved spontaneously within 1 week.

As her nevus had not changed in many years, she was instructed to continue to watch the nevus and report any changes to a physician for evaluation. If the lesion changed in the future, she should be referred to an ophthalmologist.

Correspondence
Richard P. Usatine, Editor, Photo Rounds, University of Texas Health Sciences Center at San Antonio, Dept of Family and Community Medicine, MC 7794, 7703 Floyd Curl Drive, San Antonio, TX 78229-3900. E-mail: usatine@uthscsa.edu.

An 8-year-old Hispanic girl was brought to see her family physician by her mother, who noticed 2 bald spots on the back of her daughter’s scalp while brushing her hair. The child had no itching or pain. No obvious precipitating events preceded the hair loss.

The mother was more worried than the child: she didn’t want to see her beautiful girl become bald. The girl was pleased that the bald spots could be completely covered with her long hair, but she didn’t want anyone to see them. The child was otherwise healthy. She did not have any chronic medical problems and was not taking any medications. No one else in the family had a similar problem.

When the mother lifted the hair in the back, 2 round areas of hair loss were evident (Figure 1). On close inspection, there was no scaling or scarring. Her nails were normal. The child was afebrile, and the remainder of her exam was unremarkable.

FIGURE 1
Two round areas of hair loss

What is the diagnosis?
What are the management options?

Diagnosis: alopecia areata

This is the typical appearance of alopecia areata, a chronic inflammatory disease that affects the hair follicles, causing sudden hair loss. Sometimes it affects the nails as well.¹ Alopecia areata occurs in both males and females of all ages and races.

Alopecia areata may be an autoimmune disease, though this is unproven. The affected skin may be slightly erythematous but otherwise appears normal. Short broken hairs (exclamation-mark hairs) may be seen around the margins of expanding patches of baldness. The nails are involved in about 10% of patients with severe enough alopecia to be referred to a specialist.¹

Many cases begin in childhood and can be psychologically devastating. Alopecia areata is part of a spectrum of diseases with mild to extensive hair loss (Figure 2), including alopecia totalis, in which all the hair on the scalp is lost, and alopecia universalis (Figure 3), in which all the hair on the body is lost. Extensive involvement, early age of onset, and Down syndrome are all poor prognostic factors for alopecia areata.

FIGURE 2
More extensive alopecia

FIGURE 3
Alopecia universalis

Differential diagnoses

The differential diagnosis for alopecia areata includes tinea capitis, trichotillomania, early scarring alopecia, telogen effluvium, anagen effluvium (drug-induced), systemic and discoid lupus erythematosus, and secondary syphilis. In most cases the history and physical exam are adequate to make the diagnosis.

This patient does not have the typical scalp scaling and inflammation seen with tinea capitis. Trichotillomania—hair loss caused by the purposeful pulling of hair by the patient—is likely to cause the most confusion because it coexists with alopecia areata in some cases. This child has shown no evidence of such behavior. She also has no evidence of scalp scarring as may be seen in lupus. Telogen effluvium and anagen effluvium cause a more even distribution of hair loss. The patient has no known risk factors for secondary syphilis.

Laboratory testing

No lab tests are needed in this case. If there were some scalp scaling or inflammation, a potassium hydroxide preparation of the involved area would be useful to look for fungal elements; a fungal culture might also be warranted. If needed, further investigations might include serological testing for lupus erythematosus and syphilis, and a skin biopsy if the diagnosis is still unknown.

Treatment: time, drug therapies, aromatherapy

Spontaneous remission occurs in up to 80% of patients with limited patchy hair loss of less than 1 year.¹ Spontaneous remission rates are significantly lower with more extensive hair loss.

Treatments are potentially painful, expensive, or time-consuming, and few randomized controlled trials support their use. Often the best treatment is watching for spontaneous remission.

The only adverse health effect of alopecia areata is the psychological distress that it may cause. While this is not to be taken lightly, the lack of evidence for successful treatments needs to be weighed with the patient’s ability to cope with leaving the hair loss untreated over time. In cases of extensive hair loss, the best treatment may be a wig.

For patients that have more visible and extensive areas of hair loss, the psychological impact might prompt the patient to want any treatment available despite the lack of evidence. Alopecia totalis or universalis may cause considerable psychological and social disability. Patients can be referred to the National Alopecia Areata Foundation for support groups and additional information. Individual counseling may be needed for some patients.

Steroid injections

Intralesional steroid injections may stimulate regrowth of hair at the site of injection (level of evidence [LOE]=5).¹ The effect may last a few months, but there is no evidence that it improves the long-term outcome or increases the probability of a cure. New areas of alopecia can still develop.

Injections are typically performed with 5–10 mg/mL of triamcinolone acetonide using a small-gauge needle. Most children will not be able to tolerate the scalp injections and should not be forced to endure this type of therapy even if the parent is pushing for it.

Other medications

Topical diphenylcyclopropenone (DPCP) is a contact immunotherapy that has some proven benefit with extensive alopecia areata (LOE=2b).^1,2 In 1 study, 56 patients with chronic, extensive alopecia areata (duration ranging from 1 to 10 years, involving 30% to 100% of the scalp) were treated with progressively higher concentrations of DPCP in a randomized crossover trial.² Twenty-five of 56 patients had total hair regrowth at 6 months, and no relapse occurred in 60% of patients. Side effects included local inflammation, eczema, autosensitization reaction, and eyelid edema.

Unfortunately, contact immunotherapy involves multiple visits to the office over several months, and it stimulates cosmetically worthwhile hair regrowth in <50% of patients with extensive patchy hair loss.¹ It is a reasonable alternative for patients who do not have spontaneous remission after 1 year.

While potent topical steroids and topical minoxidil are prescribed for limited patchy alopecia areata, no convincing evidence shows they are effective.¹ Likewise, no evidence warrants the use systemic steroids or psoralen/ultraviolet light treatment (PUVA).¹

Aromatherapy

The best evidence may be for aromatherapy. A single-blinded randomized controlled trial was performed with 86 patients.³ As Dr Ebell points out in his InfoPOEMs review of this study, aromatherapy involves rubbing scented essential oils into the skin to treat localized and systemic disease.⁴ Patients with alopecia areata were randomized to nightly aromatherapy—with Thymus vulgaris (thyme), Lavandula angustifolia (lavender), Rosmarinus officinalis (rosemary), and Cedrus atlantica (cedar)—or to a control consisting of carrier oils only.

Improvement was seen in 54% of the treatment group and 21% of the control group (P=.008; number needed to treat=3). Of the 19 patients in the active treatment group who reported improvement, 11 had “very good” or “excellent” improvement. The results show aromatherapy to be a safe and effective treatment for alopecia areata (LOE=1b).⁴

The main problem with this study is that the researchers did not describe the duration of the patients’ alopecia. However, in a reply to a letter, they described the patients as having had alopecia areata from less than 1 year to more than 9 years. This explains the low improvement rates in both groups but does not invalidate the statistically significant difference for those that received the essential oils.

Conclusion of visit, follow-up

For a mild case like this in which the hair loss can be hidden, the best treatment is reassurance and observation. The physician explained the natural history of the disease, including the fact that regrowth will take at least 3 months for any single patch. Therapeutic options were also discussed. The mother and child were reassured that the hair is likely to grow in on its own. Neither of them wanted intralesional injections or topical therapies.

One year later during a well-child check, it was noted that the girl’s hair had fully regrown.

An 8-year-old Hispanic girl was brought to see her family physician by her mother, who noticed 2 bald spots on the back of her daughter’s scalp while brushing her hair. The child had no itching or pain. No obvious precipitating events preceded the hair loss.

The mother was more worried than the child: she didn’t want to see her beautiful girl become bald. The girl was pleased that the bald spots could be completely covered with her long hair, but she didn’t want anyone to see them. The child was otherwise healthy. She did not have any chronic medical problems and was not taking any medications. No one else in the family had a similar problem.

When the mother lifted the hair in the back, 2 round areas of hair loss were evident (Figure 1). On close inspection, there was no scaling or scarring. Her nails were normal. The child was afebrile, and the remainder of her exam was unremarkable.

FIGURE 1
Two round areas of hair loss

What is the diagnosis?
What are the management options?

Diagnosis: alopecia areata

This is the typical appearance of alopecia areata, a chronic inflammatory disease that affects the hair follicles, causing sudden hair loss. Sometimes it affects the nails as well.¹ Alopecia areata occurs in both males and females of all ages and races.

Alopecia areata may be an autoimmune disease, though this is unproven. The affected skin may be slightly erythematous but otherwise appears normal. Short broken hairs (exclamation-mark hairs) may be seen around the margins of expanding patches of baldness. The nails are involved in about 10% of patients with severe enough alopecia to be referred to a specialist.¹

Many cases begin in childhood and can be psychologically devastating. Alopecia areata is part of a spectrum of diseases with mild to extensive hair loss (Figure 2), including alopecia totalis, in which all the hair on the scalp is lost, and alopecia universalis (Figure 3), in which all the hair on the body is lost. Extensive involvement, early age of onset, and Down syndrome are all poor prognostic factors for alopecia areata.

FIGURE 2
More extensive alopecia

FIGURE 3
Alopecia universalis

Differential diagnoses

The differential diagnosis for alopecia areata includes tinea capitis, trichotillomania, early scarring alopecia, telogen effluvium, anagen effluvium (drug-induced), systemic and discoid lupus erythematosus, and secondary syphilis. In most cases the history and physical exam are adequate to make the diagnosis.

This patient does not have the typical scalp scaling and inflammation seen with tinea capitis. Trichotillomania—hair loss caused by the purposeful pulling of hair by the patient—is likely to cause the most confusion because it coexists with alopecia areata in some cases. This child has shown no evidence of such behavior. She also has no evidence of scalp scarring as may be seen in lupus. Telogen effluvium and anagen effluvium cause a more even distribution of hair loss. The patient has no known risk factors for secondary syphilis.

Laboratory testing

No lab tests are needed in this case. If there were some scalp scaling or inflammation, a potassium hydroxide preparation of the involved area would be useful to look for fungal elements; a fungal culture might also be warranted. If needed, further investigations might include serological testing for lupus erythematosus and syphilis, and a skin biopsy if the diagnosis is still unknown.

Treatment: time, drug therapies, aromatherapy

Spontaneous remission occurs in up to 80% of patients with limited patchy hair loss of less than 1 year.¹ Spontaneous remission rates are significantly lower with more extensive hair loss.

Treatments are potentially painful, expensive, or time-consuming, and few randomized controlled trials support their use. Often the best treatment is watching for spontaneous remission.

The only adverse health effect of alopecia areata is the psychological distress that it may cause. While this is not to be taken lightly, the lack of evidence for successful treatments needs to be weighed with the patient’s ability to cope with leaving the hair loss untreated over time. In cases of extensive hair loss, the best treatment may be a wig.

For patients that have more visible and extensive areas of hair loss, the psychological impact might prompt the patient to want any treatment available despite the lack of evidence. Alopecia totalis or universalis may cause considerable psychological and social disability. Patients can be referred to the National Alopecia Areata Foundation for support groups and additional information. Individual counseling may be needed for some patients.

Steroid injections

Intralesional steroid injections may stimulate regrowth of hair at the site of injection (level of evidence [LOE]=5).¹ The effect may last a few months, but there is no evidence that it improves the long-term outcome or increases the probability of a cure. New areas of alopecia can still develop.

Injections are typically performed with 5–10 mg/mL of triamcinolone acetonide using a small-gauge needle. Most children will not be able to tolerate the scalp injections and should not be forced to endure this type of therapy even if the parent is pushing for it.

Other medications

Topical diphenylcyclopropenone (DPCP) is a contact immunotherapy that has some proven benefit with extensive alopecia areata (LOE=2b).^1,2 In 1 study, 56 patients with chronic, extensive alopecia areata (duration ranging from 1 to 10 years, involving 30% to 100% of the scalp) were treated with progressively higher concentrations of DPCP in a randomized crossover trial.² Twenty-five of 56 patients had total hair regrowth at 6 months, and no relapse occurred in 60% of patients. Side effects included local inflammation, eczema, autosensitization reaction, and eyelid edema.

Unfortunately, contact immunotherapy involves multiple visits to the office over several months, and it stimulates cosmetically worthwhile hair regrowth in <50% of patients with extensive patchy hair loss.¹ It is a reasonable alternative for patients who do not have spontaneous remission after 1 year.

While potent topical steroids and topical minoxidil are prescribed for limited patchy alopecia areata, no convincing evidence shows they are effective.¹ Likewise, no evidence warrants the use systemic steroids or psoralen/ultraviolet light treatment (PUVA).¹

Aromatherapy

The best evidence may be for aromatherapy. A single-blinded randomized controlled trial was performed with 86 patients.³ As Dr Ebell points out in his InfoPOEMs review of this study, aromatherapy involves rubbing scented essential oils into the skin to treat localized and systemic disease.⁴ Patients with alopecia areata were randomized to nightly aromatherapy—with Thymus vulgaris (thyme), Lavandula angustifolia (lavender), Rosmarinus officinalis (rosemary), and Cedrus atlantica (cedar)—or to a control consisting of carrier oils only.

Improvement was seen in 54% of the treatment group and 21% of the control group (P=.008; number needed to treat=3). Of the 19 patients in the active treatment group who reported improvement, 11 had “very good” or “excellent” improvement. The results show aromatherapy to be a safe and effective treatment for alopecia areata (LOE=1b).⁴

The main problem with this study is that the researchers did not describe the duration of the patients’ alopecia. However, in a reply to a letter, they described the patients as having had alopecia areata from less than 1 year to more than 9 years. This explains the low improvement rates in both groups but does not invalidate the statistically significant difference for those that received the essential oils.

Conclusion of visit, follow-up

For a mild case like this in which the hair loss can be hidden, the best treatment is reassurance and observation. The physician explained the natural history of the disease, including the fact that regrowth will take at least 3 months for any single patch. Therapeutic options were also discussed. The mother and child were reassured that the hair is likely to grow in on its own. Neither of them wanted intralesional injections or topical therapies.

One year later during a well-child check, it was noted that the girl’s hair had fully regrown.

An 8-year-old Hispanic girl was brought to see her family physician by her mother, who noticed 2 bald spots on the back of her daughter’s scalp while brushing her hair. The child had no itching or pain. No obvious precipitating events preceded the hair loss.

The mother was more worried than the child: she didn’t want to see her beautiful girl become bald. The girl was pleased that the bald spots could be completely covered with her long hair, but she didn’t want anyone to see them. The child was otherwise healthy. She did not have any chronic medical problems and was not taking any medications. No one else in the family had a similar problem.

When the mother lifted the hair in the back, 2 round areas of hair loss were evident (Figure 1). On close inspection, there was no scaling or scarring. Her nails were normal. The child was afebrile, and the remainder of her exam was unremarkable.

FIGURE 1
Two round areas of hair loss

What is the diagnosis?
What are the management options?

Diagnosis: alopecia areata

This is the typical appearance of alopecia areata, a chronic inflammatory disease that affects the hair follicles, causing sudden hair loss. Sometimes it affects the nails as well.¹ Alopecia areata occurs in both males and females of all ages and races.

Alopecia areata may be an autoimmune disease, though this is unproven. The affected skin may be slightly erythematous but otherwise appears normal. Short broken hairs (exclamation-mark hairs) may be seen around the margins of expanding patches of baldness. The nails are involved in about 10% of patients with severe enough alopecia to be referred to a specialist.¹

Many cases begin in childhood and can be psychologically devastating. Alopecia areata is part of a spectrum of diseases with mild to extensive hair loss (Figure 2), including alopecia totalis, in which all the hair on the scalp is lost, and alopecia universalis (Figure 3), in which all the hair on the body is lost. Extensive involvement, early age of onset, and Down syndrome are all poor prognostic factors for alopecia areata.

FIGURE 2
More extensive alopecia

FIGURE 3
Alopecia universalis

Differential diagnoses

The differential diagnosis for alopecia areata includes tinea capitis, trichotillomania, early scarring alopecia, telogen effluvium, anagen effluvium (drug-induced), systemic and discoid lupus erythematosus, and secondary syphilis. In most cases the history and physical exam are adequate to make the diagnosis.

This patient does not have the typical scalp scaling and inflammation seen with tinea capitis. Trichotillomania—hair loss caused by the purposeful pulling of hair by the patient—is likely to cause the most confusion because it coexists with alopecia areata in some cases. This child has shown no evidence of such behavior. She also has no evidence of scalp scarring as may be seen in lupus. Telogen effluvium and anagen effluvium cause a more even distribution of hair loss. The patient has no known risk factors for secondary syphilis.

Laboratory testing

No lab tests are needed in this case. If there were some scalp scaling or inflammation, a potassium hydroxide preparation of the involved area would be useful to look for fungal elements; a fungal culture might also be warranted. If needed, further investigations might include serological testing for lupus erythematosus and syphilis, and a skin biopsy if the diagnosis is still unknown.

Treatment: time, drug therapies, aromatherapy

Spontaneous remission occurs in up to 80% of patients with limited patchy hair loss of less than 1 year.¹ Spontaneous remission rates are significantly lower with more extensive hair loss.