Dermatology

In the opinion of Neil H. Shear, MD, a stepwise approach is the best way to diagnose possible drug-induced skin disease and determine the root cause.

“Often, we need to think of more than one cause,” he said during the annual meeting of the Society for Pediatric Dermatology. “It could be drug X. It could be drug Y. It could be contrast media. We must think broadly and pay special attention to skin of color, overlapping syndromes, and the changing diagnostic assessment over time.”

His suggested diagnostic triangle includes appearance of the rash or lesion(s), systemic impact, and histology. “The first is the appearance,” said Dr. Shear, professor emeritus of dermatology, clinical pharmacology and toxicology, and medicine at the University of Toronto. “Is it exanthem? Is it blistering? Don’t just say drug ‘rash.’ That doesn’t work. You need to know if there are systemic features, and sometimes histologic information can change your approach or diagnosis, but not as often as one might think,” he said, noting that, in his view, the two main factors are appearance and systemic impact.

The presence of fever is a hallmark of systemic problems, he continued, “so if you see fever, you know you’re probably going to be dealing with a complex reaction, so we need to know the morphology.” Consider whether it is simple exanthem (a mild, uncomplicated rash) or complex exanthem (drug rash with eosinophilia and systemic symptoms or fever, malaise, and adenopathy).

As for other morphologies, urticarial lesions could be urticaria or a serum sickness-like reaction, pustular lesions could be acneiform or acute generalized exanthematous pustulosis, while blistering lesions could suggest a fixed drug response or Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN).

Dr. Shear considers SJS/TEN as a spectrum of blistering disease, “because there’s not a single diagnosis,” he said. “There’s a spectrum, if you will, depending on how advanced people are in their disease.” He coauthored a 1991 report describing eight cases of mycoplasma and Stevens-Johnson syndrome. “I was surprised at how long that stood up as about the only paper in that area,” he said. “But there’s much more happening now with a proliferation of terms,” he added, referring to MIRM (Mycoplasma pneumonia–induced rash and mucositis), RIME (reactive infectious mucocutaneous eruption); and Fuchs syndrome, or SJS without skin lesions.

What was not appreciated in the early classification of SJS, he continued, was a “side basket” of bullous erythema multiforme. “We didn’t know what to call it,” he said. “At one point we called it bullous erythema multiforme. At another point we called it erythema multiforme major. We just didn’t know what it was.”

The appearance and systemic effects of SJS comprise what he termed SJS type 2 – or the early stages of TEN. Taken together, he refers to these two conditions as TEN Spectrum, or TENS. “One of the traps is that TENS can look like varicella, and vice versa, especially in very dark brown or black skin,” Dr. Shear said. “You have to be careful. A biopsy might be worthwhile. Acute lupus has the pathology of TENS but the patients are not as systemically ill as true TENS.”

In 2011, Japanese researchers reported on 38 cases of SJS associated with M. pneumoniae, and 78 cases of drug-induced SJS. They found that 66% of adult patients with M. pneumoniae–associated SJS developed mucocutaneous lesions and fever/respiratory symptoms on the same day, mostly shortness of breath and cough. In contrast, most of the patients aged under 20 years developed fever/respiratory symptoms before mucocutaneous involvement.

“The big clinical differentiator between drug-induced SJS and mycoplasma-induced SJS was respiratory disorder,” said Dr. Shear, who was not affiliated with the study. “That means you’re probably looking at something that’s mycoplasma related [when respiratory problems are present]. Even if you can’t prove it’s mycoplasma related, that probably needs to be the target of your therapy. The idea ... is to make sure it’s clear at the end. One, so they get better, and two, so that we’re not giving drugs needlessly when it was really mycoplasma.”

Noting that HLA-B*15:02 is a marker for carbamazepine-induced SJS and TEN, he said, “a positive HLA test can support the diagnosis, confirm the suspected offending drug, and is valuable for familial genetic counseling.”

As for treatment of SJS, TEN, and other cytotoxic T-lymphocyte–mediated severe cutaneous adverse reactions, a randomized Japanese clinical trial evaluating prednisolone 1-1.5 mg/kg/day IV versus etanercept 25-50 mg subcutaneously twice per week in 96 patients with SJS-TEN found that etanercept decreased the mortality rate by 8.3%. In addition, etanercept reduced skin healing time, when compared with prednisolone (a median of 14 vs. 19 days, respectively; P = .010), and was associated with a lower incidence of GI hemorrhage (2.6% vs. 18.2%, respectively; P = .03).

Dr. Shear said that he would like to see better therapeutics for severe, complex patients. “After leaving the hospital, people with SJS or people with TEN need to have ongoing care, consultation, and explanation so they and their families know what drugs are safe in the future.”

Dr. Shear disclosed that he has been a consultant to AbbVie, Amgen, Bausch Medicine, Novartis, Sanofi-Genzyme, UCB, LEO Pharma, Otsuka, Janssen, Alpha Laboratories, Lilly, ChemoCentryx, Vivoryon, Galderma, Innovaderm, Chromocell, and Kyowa Kirin.

dbrunk@mdedge.com

In the opinion of Neil H. Shear, MD, a stepwise approach is the best way to diagnose possible drug-induced skin disease and determine the root cause.

“Often, we need to think of more than one cause,” he said during the annual meeting of the Society for Pediatric Dermatology. “It could be drug X. It could be drug Y. It could be contrast media. We must think broadly and pay special attention to skin of color, overlapping syndromes, and the changing diagnostic assessment over time.”

His suggested diagnostic triangle includes appearance of the rash or lesion(s), systemic impact, and histology. “The first is the appearance,” said Dr. Shear, professor emeritus of dermatology, clinical pharmacology and toxicology, and medicine at the University of Toronto. “Is it exanthem? Is it blistering? Don’t just say drug ‘rash.’ That doesn’t work. You need to know if there are systemic features, and sometimes histologic information can change your approach or diagnosis, but not as often as one might think,” he said, noting that, in his view, the two main factors are appearance and systemic impact.

The presence of fever is a hallmark of systemic problems, he continued, “so if you see fever, you know you’re probably going to be dealing with a complex reaction, so we need to know the morphology.” Consider whether it is simple exanthem (a mild, uncomplicated rash) or complex exanthem (drug rash with eosinophilia and systemic symptoms or fever, malaise, and adenopathy).

As for other morphologies, urticarial lesions could be urticaria or a serum sickness-like reaction, pustular lesions could be acneiform or acute generalized exanthematous pustulosis, while blistering lesions could suggest a fixed drug response or Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN).

Dr. Shear considers SJS/TEN as a spectrum of blistering disease, “because there’s not a single diagnosis,” he said. “There’s a spectrum, if you will, depending on how advanced people are in their disease.” He coauthored a 1991 report describing eight cases of mycoplasma and Stevens-Johnson syndrome. “I was surprised at how long that stood up as about the only paper in that area,” he said. “But there’s much more happening now with a proliferation of terms,” he added, referring to MIRM (Mycoplasma pneumonia–induced rash and mucositis), RIME (reactive infectious mucocutaneous eruption); and Fuchs syndrome, or SJS without skin lesions.

What was not appreciated in the early classification of SJS, he continued, was a “side basket” of bullous erythema multiforme. “We didn’t know what to call it,” he said. “At one point we called it bullous erythema multiforme. At another point we called it erythema multiforme major. We just didn’t know what it was.”

The appearance and systemic effects of SJS comprise what he termed SJS type 2 – or the early stages of TEN. Taken together, he refers to these two conditions as TEN Spectrum, or TENS. “One of the traps is that TENS can look like varicella, and vice versa, especially in very dark brown or black skin,” Dr. Shear said. “You have to be careful. A biopsy might be worthwhile. Acute lupus has the pathology of TENS but the patients are not as systemically ill as true TENS.”

In 2011, Japanese researchers reported on 38 cases of SJS associated with M. pneumoniae, and 78 cases of drug-induced SJS. They found that 66% of adult patients with M. pneumoniae–associated SJS developed mucocutaneous lesions and fever/respiratory symptoms on the same day, mostly shortness of breath and cough. In contrast, most of the patients aged under 20 years developed fever/respiratory symptoms before mucocutaneous involvement.

“The big clinical differentiator between drug-induced SJS and mycoplasma-induced SJS was respiratory disorder,” said Dr. Shear, who was not affiliated with the study. “That means you’re probably looking at something that’s mycoplasma related [when respiratory problems are present]. Even if you can’t prove it’s mycoplasma related, that probably needs to be the target of your therapy. The idea ... is to make sure it’s clear at the end. One, so they get better, and two, so that we’re not giving drugs needlessly when it was really mycoplasma.”

Noting that HLA-B*15:02 is a marker for carbamazepine-induced SJS and TEN, he said, “a positive HLA test can support the diagnosis, confirm the suspected offending drug, and is valuable for familial genetic counseling.”

As for treatment of SJS, TEN, and other cytotoxic T-lymphocyte–mediated severe cutaneous adverse reactions, a randomized Japanese clinical trial evaluating prednisolone 1-1.5 mg/kg/day IV versus etanercept 25-50 mg subcutaneously twice per week in 96 patients with SJS-TEN found that etanercept decreased the mortality rate by 8.3%. In addition, etanercept reduced skin healing time, when compared with prednisolone (a median of 14 vs. 19 days, respectively; P = .010), and was associated with a lower incidence of GI hemorrhage (2.6% vs. 18.2%, respectively; P = .03).

Dr. Shear said that he would like to see better therapeutics for severe, complex patients. “After leaving the hospital, people with SJS or people with TEN need to have ongoing care, consultation, and explanation so they and their families know what drugs are safe in the future.”

Dr. Shear disclosed that he has been a consultant to AbbVie, Amgen, Bausch Medicine, Novartis, Sanofi-Genzyme, UCB, LEO Pharma, Otsuka, Janssen, Alpha Laboratories, Lilly, ChemoCentryx, Vivoryon, Galderma, Innovaderm, Chromocell, and Kyowa Kirin.

dbrunk@mdedge.com

In the opinion of Neil H. Shear, MD, a stepwise approach is the best way to diagnose possible drug-induced skin disease and determine the root cause.

“Often, we need to think of more than one cause,” he said during the annual meeting of the Society for Pediatric Dermatology. “It could be drug X. It could be drug Y. It could be contrast media. We must think broadly and pay special attention to skin of color, overlapping syndromes, and the changing diagnostic assessment over time.”

His suggested diagnostic triangle includes appearance of the rash or lesion(s), systemic impact, and histology. “The first is the appearance,” said Dr. Shear, professor emeritus of dermatology, clinical pharmacology and toxicology, and medicine at the University of Toronto. “Is it exanthem? Is it blistering? Don’t just say drug ‘rash.’ That doesn’t work. You need to know if there are systemic features, and sometimes histologic information can change your approach or diagnosis, but not as often as one might think,” he said, noting that, in his view, the two main factors are appearance and systemic impact.

The presence of fever is a hallmark of systemic problems, he continued, “so if you see fever, you know you’re probably going to be dealing with a complex reaction, so we need to know the morphology.” Consider whether it is simple exanthem (a mild, uncomplicated rash) or complex exanthem (drug rash with eosinophilia and systemic symptoms or fever, malaise, and adenopathy).

As for other morphologies, urticarial lesions could be urticaria or a serum sickness-like reaction, pustular lesions could be acneiform or acute generalized exanthematous pustulosis, while blistering lesions could suggest a fixed drug response or Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN).

Dr. Shear considers SJS/TEN as a spectrum of blistering disease, “because there’s not a single diagnosis,” he said. “There’s a spectrum, if you will, depending on how advanced people are in their disease.” He coauthored a 1991 report describing eight cases of mycoplasma and Stevens-Johnson syndrome. “I was surprised at how long that stood up as about the only paper in that area,” he said. “But there’s much more happening now with a proliferation of terms,” he added, referring to MIRM (Mycoplasma pneumonia–induced rash and mucositis), RIME (reactive infectious mucocutaneous eruption); and Fuchs syndrome, or SJS without skin lesions.

What was not appreciated in the early classification of SJS, he continued, was a “side basket” of bullous erythema multiforme. “We didn’t know what to call it,” he said. “At one point we called it bullous erythema multiforme. At another point we called it erythema multiforme major. We just didn’t know what it was.”

The appearance and systemic effects of SJS comprise what he termed SJS type 2 – or the early stages of TEN. Taken together, he refers to these two conditions as TEN Spectrum, or TENS. “One of the traps is that TENS can look like varicella, and vice versa, especially in very dark brown or black skin,” Dr. Shear said. “You have to be careful. A biopsy might be worthwhile. Acute lupus has the pathology of TENS but the patients are not as systemically ill as true TENS.”

In 2011, Japanese researchers reported on 38 cases of SJS associated with M. pneumoniae, and 78 cases of drug-induced SJS. They found that 66% of adult patients with M. pneumoniae–associated SJS developed mucocutaneous lesions and fever/respiratory symptoms on the same day, mostly shortness of breath and cough. In contrast, most of the patients aged under 20 years developed fever/respiratory symptoms before mucocutaneous involvement.

“The big clinical differentiator between drug-induced SJS and mycoplasma-induced SJS was respiratory disorder,” said Dr. Shear, who was not affiliated with the study. “That means you’re probably looking at something that’s mycoplasma related [when respiratory problems are present]. Even if you can’t prove it’s mycoplasma related, that probably needs to be the target of your therapy. The idea ... is to make sure it’s clear at the end. One, so they get better, and two, so that we’re not giving drugs needlessly when it was really mycoplasma.”

Noting that HLA-B*15:02 is a marker for carbamazepine-induced SJS and TEN, he said, “a positive HLA test can support the diagnosis, confirm the suspected offending drug, and is valuable for familial genetic counseling.”

As for treatment of SJS, TEN, and other cytotoxic T-lymphocyte–mediated severe cutaneous adverse reactions, a randomized Japanese clinical trial evaluating prednisolone 1-1.5 mg/kg/day IV versus etanercept 25-50 mg subcutaneously twice per week in 96 patients with SJS-TEN found that etanercept decreased the mortality rate by 8.3%. In addition, etanercept reduced skin healing time, when compared with prednisolone (a median of 14 vs. 19 days, respectively; P = .010), and was associated with a lower incidence of GI hemorrhage (2.6% vs. 18.2%, respectively; P = .03).

Dr. Shear said that he would like to see better therapeutics for severe, complex patients. “After leaving the hospital, people with SJS or people with TEN need to have ongoing care, consultation, and explanation so they and their families know what drugs are safe in the future.”

Dr. Shear disclosed that he has been a consultant to AbbVie, Amgen, Bausch Medicine, Novartis, Sanofi-Genzyme, UCB, LEO Pharma, Otsuka, Janssen, Alpha Laboratories, Lilly, ChemoCentryx, Vivoryon, Galderma, Innovaderm, Chromocell, and Kyowa Kirin.

dbrunk@mdedge.com

Five of the more uncommon dermatologic disorders – sarcoidosis, Rosai-Dorfman disease, Erdheim-Chester disease, eosinophilic fasciitis, and cutaneous Crohn disease – are linked to internal diseases and may spawn misdiagnoses, a dermatologist told colleagues.

“Proper diagnosis can lead to an effective management in our patients,” said Jeffrey Callen, MD, professor of medicine and chief of dermatology at the University of Louisville (Ky.), who spoke at the Inaugural Symposium for Inflammatory Skin Disease.
 

Sarcoidosis

The cause of sarcoidosis, an inflammatory disease that tends to affect the lungs, “is unknown, but it’s probably an immunologic disorder,” Dr. Callen said, “and there probably is a genetic predisposition.” About 20%-25% of patients with sarcoidosis have skin lesions that are either “specific” (a biopsy that reveals a noncaseating – “naked” – granuloma) or “nonspecific” (most commonly, erythema nodosum, or EN).

The specific lesions in sarcoidosis may occur in parts of the body, such as the knees, which were injured earlier in life and may have taken in foreign bodies, Dr. Callen said. As for nonspecific lesions, about 20% of patients with EN have an acute, self-limiting form of sarcoidosis. “These patients will have bilateral hilar lymphadenopathy, anterior uveitis, and polyarthritis. It’s generally treated symptomatically because it goes away on its own.”

He cautioned colleagues to beware of indurated, infiltrative facial lesions known as lupus pernio that are commonly found on the nose. They’re more prevalent in Black patients and possibly women, who are at higher risk of manifestations outside the skin, he said. “If you have it along the nasal rim, you should look into the upper respiratory tract for involvement.”

Dr. Callen recommends an extensive workup in patients with suspected sarcoidosis, including biopsy (with the exception of EN lesions), cultures and special stains, and screening when appropriate, for disease in organs such as the eyes, lungs, heart, and kidneys.

As for treatment, “the disease is in the dermis, and some topical therapies are not highly effective,” he said. There are injections that can be given, including corticosteroids, and there are a variety of oral treatments that are all off label.” These include corticosteroids, antimalarials, allopurinol, and tetracyclines, among several others. Subcutaneous and intravenous treatments are also options, along with surgery and laser therapy to treat specific lesions.

Rosai-Dorfman disease

This rare disorder is caused by overproduction of certain white blood cells in the lymph nodes, which can cause nodular lesions. The disease most often appears in children and young adults, often Black individuals and males. It is fatal in as many as 11% of patients, justifying aggressive treatment in patients with aggressive disease, Dr. Callen said. When it’s limited to the skin, however, “nothing may need to be done.”

Dr. Callen highlighted consensus recommendations about diagnosis and treatment of Rosai-Dorfman disease published in 2018.

He also noted the existence of cutaneous Rosai-Dorfman disease, a “solitary process” that appears more commonly in females, and in people of Asian heritage, compared with White individuals. It is characterized by single, clustered or widespread lesions: They can be xanthomatous, erythematous, or red-brown papules, nodules, and plaques. They’re acneiform, pustular, giant granuloma annulare–like, subcutaneous, and vasculitis-like, he said.

While Rosai-Dorfman disease can be linked to lymphoma, hypothyroidism, and lupus erythematosus, “nothing necessarily needs to be done when it’s skin-limited since it can be self-resolving,” he noted. Other treatments include radiotherapy, cryotherapy, excision, topical and oral corticosteroids, thalidomide, and methotrexate.

The disease can be serious, and is fatal in 5% of cases. When a vital organ is threatened, Dr. Callen suggested surgery, chemotherapy, or radiation.
 

Erdheim-Chester disease

This disease – which is extremely rare, with just 500 cases noted before 2014 – occurs when the body overproduces macrophages. It’s most common in middle-aged people and in men, who make up 75% of cases. About a quarter of patients develop skin lesions: Red-brown to yellow nodules and xanthelasma-like indurated plaques on the eyelids, scalp, neck, trunk, and axillae, and “other cutaneous manifestations have been reported in patients,” Dr. Callen said.

The disease also frequently affects the bones, large vessels, heart, lungs, and central nervous system. Interferon-alpha is the first-line treatment, and there are several other alternative therapies, although 5-year survival (68%) is poor, and it is especially likely to be fatal in those with central nervous system involvement.
 

Eosinophilic fasciitis

Eosinophilic fasciitis (EF) “is a disorder of unknown etiology that causes sclerosis of the skin” without Raynaud’s phenomenon, Dr. Callen said. Look for erythema, swelling, and induration of the extremities that is accompanied by peripheral eosinophilia, and if necessary, confirm the diagnosis with full skin-to-muscle biopsy or MRI.

There are many possible triggers, including strenuous exercise, initiation with hemodialysis, radiation therapy and burns, and graft-versus-host disease. Other potential causes include exposure to medications such as statins, phenytoin, ramipril, subcutaneous heparin, and immune checkpoint inhibitor therapy. The disorder is also linked to autoimmune and hematologic disorders.

Dr. Callen, who highlighted EF guidelines published in 2018, said treatments include physical therapy, prednisone, methotrexate, mycophenolate, and hydroxychloroquine.
 

Metastatic Crohn’s disease

This is a rare granulomatous inflammation of skin that often affects the genitals, especially in children. It is noncontiguous with the GI tract, and severity of skin involvement does not always parallel the severity of the disease in the GI tract, Dr. Callen said. However, the condition can occur before or simultaneously with the development of GI disease, or after GI surgery.

He highlighted a review of metastatic Crohn’s disease, published in 2014, and noted that there are multiple treatments, including systemic corticosteroids, tumor necrosis factor–alpha inhibitors, and topical therapies.

Dr. Callen reported no relevant disclosures.

Five of the more uncommon dermatologic disorders – sarcoidosis, Rosai-Dorfman disease, Erdheim-Chester disease, eosinophilic fasciitis, and cutaneous Crohn disease – are linked to internal diseases and may spawn misdiagnoses, a dermatologist told colleagues.

“Proper diagnosis can lead to an effective management in our patients,” said Jeffrey Callen, MD, professor of medicine and chief of dermatology at the University of Louisville (Ky.), who spoke at the Inaugural Symposium for Inflammatory Skin Disease.
 

Sarcoidosis

The cause of sarcoidosis, an inflammatory disease that tends to affect the lungs, “is unknown, but it’s probably an immunologic disorder,” Dr. Callen said, “and there probably is a genetic predisposition.” About 20%-25% of patients with sarcoidosis have skin lesions that are either “specific” (a biopsy that reveals a noncaseating – “naked” – granuloma) or “nonspecific” (most commonly, erythema nodosum, or EN).

The specific lesions in sarcoidosis may occur in parts of the body, such as the knees, which were injured earlier in life and may have taken in foreign bodies, Dr. Callen said. As for nonspecific lesions, about 20% of patients with EN have an acute, self-limiting form of sarcoidosis. “These patients will have bilateral hilar lymphadenopathy, anterior uveitis, and polyarthritis. It’s generally treated symptomatically because it goes away on its own.”

He cautioned colleagues to beware of indurated, infiltrative facial lesions known as lupus pernio that are commonly found on the nose. They’re more prevalent in Black patients and possibly women, who are at higher risk of manifestations outside the skin, he said. “If you have it along the nasal rim, you should look into the upper respiratory tract for involvement.”

Dr. Callen recommends an extensive workup in patients with suspected sarcoidosis, including biopsy (with the exception of EN lesions), cultures and special stains, and screening when appropriate, for disease in organs such as the eyes, lungs, heart, and kidneys.

As for treatment, “the disease is in the dermis, and some topical therapies are not highly effective,” he said. There are injections that can be given, including corticosteroids, and there are a variety of oral treatments that are all off label.” These include corticosteroids, antimalarials, allopurinol, and tetracyclines, among several others. Subcutaneous and intravenous treatments are also options, along with surgery and laser therapy to treat specific lesions.

Rosai-Dorfman disease

This rare disorder is caused by overproduction of certain white blood cells in the lymph nodes, which can cause nodular lesions. The disease most often appears in children and young adults, often Black individuals and males. It is fatal in as many as 11% of patients, justifying aggressive treatment in patients with aggressive disease, Dr. Callen said. When it’s limited to the skin, however, “nothing may need to be done.”

Dr. Callen highlighted consensus recommendations about diagnosis and treatment of Rosai-Dorfman disease published in 2018.

He also noted the existence of cutaneous Rosai-Dorfman disease, a “solitary process” that appears more commonly in females, and in people of Asian heritage, compared with White individuals. It is characterized by single, clustered or widespread lesions: They can be xanthomatous, erythematous, or red-brown papules, nodules, and plaques. They’re acneiform, pustular, giant granuloma annulare–like, subcutaneous, and vasculitis-like, he said.

While Rosai-Dorfman disease can be linked to lymphoma, hypothyroidism, and lupus erythematosus, “nothing necessarily needs to be done when it’s skin-limited since it can be self-resolving,” he noted. Other treatments include radiotherapy, cryotherapy, excision, topical and oral corticosteroids, thalidomide, and methotrexate.

The disease can be serious, and is fatal in 5% of cases. When a vital organ is threatened, Dr. Callen suggested surgery, chemotherapy, or radiation.
 

Erdheim-Chester disease

This disease – which is extremely rare, with just 500 cases noted before 2014 – occurs when the body overproduces macrophages. It’s most common in middle-aged people and in men, who make up 75% of cases. About a quarter of patients develop skin lesions: Red-brown to yellow nodules and xanthelasma-like indurated plaques on the eyelids, scalp, neck, trunk, and axillae, and “other cutaneous manifestations have been reported in patients,” Dr. Callen said.

The disease also frequently affects the bones, large vessels, heart, lungs, and central nervous system. Interferon-alpha is the first-line treatment, and there are several other alternative therapies, although 5-year survival (68%) is poor, and it is especially likely to be fatal in those with central nervous system involvement.
 

Eosinophilic fasciitis

Eosinophilic fasciitis (EF) “is a disorder of unknown etiology that causes sclerosis of the skin” without Raynaud’s phenomenon, Dr. Callen said. Look for erythema, swelling, and induration of the extremities that is accompanied by peripheral eosinophilia, and if necessary, confirm the diagnosis with full skin-to-muscle biopsy or MRI.

There are many possible triggers, including strenuous exercise, initiation with hemodialysis, radiation therapy and burns, and graft-versus-host disease. Other potential causes include exposure to medications such as statins, phenytoin, ramipril, subcutaneous heparin, and immune checkpoint inhibitor therapy. The disorder is also linked to autoimmune and hematologic disorders.

Dr. Callen, who highlighted EF guidelines published in 2018, said treatments include physical therapy, prednisone, methotrexate, mycophenolate, and hydroxychloroquine.
 

Metastatic Crohn’s disease

This is a rare granulomatous inflammation of skin that often affects the genitals, especially in children. It is noncontiguous with the GI tract, and severity of skin involvement does not always parallel the severity of the disease in the GI tract, Dr. Callen said. However, the condition can occur before or simultaneously with the development of GI disease, or after GI surgery.

He highlighted a review of metastatic Crohn’s disease, published in 2014, and noted that there are multiple treatments, including systemic corticosteroids, tumor necrosis factor–alpha inhibitors, and topical therapies.

Dr. Callen reported no relevant disclosures.

Five of the more uncommon dermatologic disorders – sarcoidosis, Rosai-Dorfman disease, Erdheim-Chester disease, eosinophilic fasciitis, and cutaneous Crohn disease – are linked to internal diseases and may spawn misdiagnoses, a dermatologist told colleagues.

“Proper diagnosis can lead to an effective management in our patients,” said Jeffrey Callen, MD, professor of medicine and chief of dermatology at the University of Louisville (Ky.), who spoke at the Inaugural Symposium for Inflammatory Skin Disease.
 

Sarcoidosis

The cause of sarcoidosis, an inflammatory disease that tends to affect the lungs, “is unknown, but it’s probably an immunologic disorder,” Dr. Callen said, “and there probably is a genetic predisposition.” About 20%-25% of patients with sarcoidosis have skin lesions that are either “specific” (a biopsy that reveals a noncaseating – “naked” – granuloma) or “nonspecific” (most commonly, erythema nodosum, or EN).

The specific lesions in sarcoidosis may occur in parts of the body, such as the knees, which were injured earlier in life and may have taken in foreign bodies, Dr. Callen said. As for nonspecific lesions, about 20% of patients with EN have an acute, self-limiting form of sarcoidosis. “These patients will have bilateral hilar lymphadenopathy, anterior uveitis, and polyarthritis. It’s generally treated symptomatically because it goes away on its own.”

He cautioned colleagues to beware of indurated, infiltrative facial lesions known as lupus pernio that are commonly found on the nose. They’re more prevalent in Black patients and possibly women, who are at higher risk of manifestations outside the skin, he said. “If you have it along the nasal rim, you should look into the upper respiratory tract for involvement.”

Dr. Callen recommends an extensive workup in patients with suspected sarcoidosis, including biopsy (with the exception of EN lesions), cultures and special stains, and screening when appropriate, for disease in organs such as the eyes, lungs, heart, and kidneys.

As for treatment, “the disease is in the dermis, and some topical therapies are not highly effective,” he said. There are injections that can be given, including corticosteroids, and there are a variety of oral treatments that are all off label.” These include corticosteroids, antimalarials, allopurinol, and tetracyclines, among several others. Subcutaneous and intravenous treatments are also options, along with surgery and laser therapy to treat specific lesions.

Rosai-Dorfman disease

This rare disorder is caused by overproduction of certain white blood cells in the lymph nodes, which can cause nodular lesions. The disease most often appears in children and young adults, often Black individuals and males. It is fatal in as many as 11% of patients, justifying aggressive treatment in patients with aggressive disease, Dr. Callen said. When it’s limited to the skin, however, “nothing may need to be done.”

Dr. Callen highlighted consensus recommendations about diagnosis and treatment of Rosai-Dorfman disease published in 2018.

He also noted the existence of cutaneous Rosai-Dorfman disease, a “solitary process” that appears more commonly in females, and in people of Asian heritage, compared with White individuals. It is characterized by single, clustered or widespread lesions: They can be xanthomatous, erythematous, or red-brown papules, nodules, and plaques. They’re acneiform, pustular, giant granuloma annulare–like, subcutaneous, and vasculitis-like, he said.

While Rosai-Dorfman disease can be linked to lymphoma, hypothyroidism, and lupus erythematosus, “nothing necessarily needs to be done when it’s skin-limited since it can be self-resolving,” he noted. Other treatments include radiotherapy, cryotherapy, excision, topical and oral corticosteroids, thalidomide, and methotrexate.

The disease can be serious, and is fatal in 5% of cases. When a vital organ is threatened, Dr. Callen suggested surgery, chemotherapy, or radiation.
 

Erdheim-Chester disease

This disease – which is extremely rare, with just 500 cases noted before 2014 – occurs when the body overproduces macrophages. It’s most common in middle-aged people and in men, who make up 75% of cases. About a quarter of patients develop skin lesions: Red-brown to yellow nodules and xanthelasma-like indurated plaques on the eyelids, scalp, neck, trunk, and axillae, and “other cutaneous manifestations have been reported in patients,” Dr. Callen said.

The disease also frequently affects the bones, large vessels, heart, lungs, and central nervous system. Interferon-alpha is the first-line treatment, and there are several other alternative therapies, although 5-year survival (68%) is poor, and it is especially likely to be fatal in those with central nervous system involvement.
 

Eosinophilic fasciitis

Eosinophilic fasciitis (EF) “is a disorder of unknown etiology that causes sclerosis of the skin” without Raynaud’s phenomenon, Dr. Callen said. Look for erythema, swelling, and induration of the extremities that is accompanied by peripheral eosinophilia, and if necessary, confirm the diagnosis with full skin-to-muscle biopsy or MRI.

There are many possible triggers, including strenuous exercise, initiation with hemodialysis, radiation therapy and burns, and graft-versus-host disease. Other potential causes include exposure to medications such as statins, phenytoin, ramipril, subcutaneous heparin, and immune checkpoint inhibitor therapy. The disorder is also linked to autoimmune and hematologic disorders.

Dr. Callen, who highlighted EF guidelines published in 2018, said treatments include physical therapy, prednisone, methotrexate, mycophenolate, and hydroxychloroquine.
 

Metastatic Crohn’s disease

This is a rare granulomatous inflammation of skin that often affects the genitals, especially in children. It is noncontiguous with the GI tract, and severity of skin involvement does not always parallel the severity of the disease in the GI tract, Dr. Callen said. However, the condition can occur before or simultaneously with the development of GI disease, or after GI surgery.

He highlighted a review of metastatic Crohn’s disease, published in 2014, and noted that there are multiple treatments, including systemic corticosteroids, tumor necrosis factor–alpha inhibitors, and topical therapies.

Dr. Callen reported no relevant disclosures.

The color change in this case is subtle, but consistent with green nail syndrome also termed chloronychia.

Green nail syndrome is a form of chronic paronychia caused by Pseudomonas aeruginosa and is associated with frequent water exposure. Pseudomonas expresses pyocyanin and pyoverdine, leading to the characteristic blue or green coloration. A Woods lamp may reveal neon green fluorescence.

Risk factors include frequent water exposure, use of an instrument to relieve symptoms of an ingrown nail, and chronic immunosuppression. In this case, frequent hand washing being performed by this patient during the COVID-19 pandemic was the likely culprit.

The differential diagnosis includes simple structural onycholysis, onychomycosis, and nail psoriasis. Polymicrobial infection can occur. When in doubt, culture or pathologic examination of nail clippings with Grocott methenamine silver and periodic acid–Schiff can identify coincident fungal infection.¹ While systemic pseudomonas infection could also occur with green nail syndrome, the latter is usually only a mild nuisance and not associated with severe disease.

Treatment consists of avoidance of water and instrumentation. Topical sodium hypochlorite 2% solution applied to the nail or acetic acid soaks for 2 to 3 weeks is often curative. Antibiotic therapy may also be used. Topical tobramycin or gentamicin are available as otic or ophthalmic drops and can be applied just under the nail.

The patient was advised to meticulously dry her hands after washing them and to apply 1 drop of topical gentamicin solution 0.3% tid for 3 weeks. The green discoloration cleared in 3 weeks and the onycholysis resolved within 4 months.

‌

Text courtesy of Jonathan Karnes, MD, medical director, MDFMR Dermatology Services, Augusta, ME. Photos courtesy of Jonathan Karnes, MD (copyright retained).

The color change in this case is subtle, but consistent with green nail syndrome also termed chloronychia.

Green nail syndrome is a form of chronic paronychia caused by Pseudomonas aeruginosa and is associated with frequent water exposure. Pseudomonas expresses pyocyanin and pyoverdine, leading to the characteristic blue or green coloration. A Woods lamp may reveal neon green fluorescence.

Risk factors include frequent water exposure, use of an instrument to relieve symptoms of an ingrown nail, and chronic immunosuppression. In this case, frequent hand washing being performed by this patient during the COVID-19 pandemic was the likely culprit.

The differential diagnosis includes simple structural onycholysis, onychomycosis, and nail psoriasis. Polymicrobial infection can occur. When in doubt, culture or pathologic examination of nail clippings with Grocott methenamine silver and periodic acid–Schiff can identify coincident fungal infection.¹ While systemic pseudomonas infection could also occur with green nail syndrome, the latter is usually only a mild nuisance and not associated with severe disease.

Treatment consists of avoidance of water and instrumentation. Topical sodium hypochlorite 2% solution applied to the nail or acetic acid soaks for 2 to 3 weeks is often curative. Antibiotic therapy may also be used. Topical tobramycin or gentamicin are available as otic or ophthalmic drops and can be applied just under the nail.

The patient was advised to meticulously dry her hands after washing them and to apply 1 drop of topical gentamicin solution 0.3% tid for 3 weeks. The green discoloration cleared in 3 weeks and the onycholysis resolved within 4 months.

‌

Text courtesy of Jonathan Karnes, MD, medical director, MDFMR Dermatology Services, Augusta, ME. Photos courtesy of Jonathan Karnes, MD (copyright retained).

The color change in this case is subtle, but consistent with green nail syndrome also termed chloronychia.

Green nail syndrome is a form of chronic paronychia caused by Pseudomonas aeruginosa and is associated with frequent water exposure. Pseudomonas expresses pyocyanin and pyoverdine, leading to the characteristic blue or green coloration. A Woods lamp may reveal neon green fluorescence.

Risk factors include frequent water exposure, use of an instrument to relieve symptoms of an ingrown nail, and chronic immunosuppression. In this case, frequent hand washing being performed by this patient during the COVID-19 pandemic was the likely culprit.

The differential diagnosis includes simple structural onycholysis, onychomycosis, and nail psoriasis. Polymicrobial infection can occur. When in doubt, culture or pathologic examination of nail clippings with Grocott methenamine silver and periodic acid–Schiff can identify coincident fungal infection.¹ While systemic pseudomonas infection could also occur with green nail syndrome, the latter is usually only a mild nuisance and not associated with severe disease.

Treatment consists of avoidance of water and instrumentation. Topical sodium hypochlorite 2% solution applied to the nail or acetic acid soaks for 2 to 3 weeks is often curative. Antibiotic therapy may also be used. Topical tobramycin or gentamicin are available as otic or ophthalmic drops and can be applied just under the nail.

The patient was advised to meticulously dry her hands after washing them and to apply 1 drop of topical gentamicin solution 0.3% tid for 3 weeks. The green discoloration cleared in 3 weeks and the onycholysis resolved within 4 months.

‌

Text courtesy of Jonathan Karnes, MD, medical director, MDFMR Dermatology Services, Augusta, ME. Photos courtesy of Jonathan Karnes, MD (copyright retained).

The Food and Drug Administration has approved Octagam 10% as the first intravenous immunoglobulin with an indication specifically for adult dermatomyositis, according to a statement from manufacturer Octapharma USA.

Dermatomyositis is a rare, idiopathic autoimmune disorder that affects approximately 10 out of every million people in the United States, mainly adults in their late 40s to early 60s, according to the company, but children aged 5-15 years can be affected. The disease is characterized by skin rashes, chronic muscle inflammation, progressive muscle weakness, and risk for mortality that is three times higher than for the general population.

There are no previously approved treatments for dermatomyositis prior to Octagam 10%, which also is indicated for chronic immune thrombocytopenic purpura in adults.

The approval for dermatomyositis was based on the results of a phase 3 randomized, double-blind, placebo-controlled clinical trial (the ProDERM trial) that included 95 adult patients at 36 sites worldwide, with 17 sites in the United States. In the trial, 78.7% of patients with dermatomyositis who were randomized to receive 2 g/kg of Octagam 10% every 4 weeks showed response at 16 weeks, compared with 43.8% of patients who received placebo. Response was based on the 2016 American College of Rheumatology/European Alliance of Associations for Rheumatology myositis response criteria. Placebo patients who switched to intravenous immunoglobulin (IVIG) during a trial extension had response rates at week 40 similar to the original patients at week 16.

“The study gives clinicians much more confidence in the efficacy and safety of intravenous immunoglobulin and provides valuable information about what type of patient is best suited for the treatment,” Rohit Aggarwal, MD, medical director of the Arthritis and Autoimmunity Center at the University of Pittsburgh and a member of the ProDERM study Steering Committee, said in the Octapharma statement.

Safety and tolerability were similar to profiles seen with other IVIG medications, according to the statement. The medication does carry a boxed warning from its chronic ITP approval, cautioning about the potential for thrombosis, renal dysfunction, and acute renal failure.

The most common adverse reactions reported by dermatomyositis patients in the ProDERM trial were headache, fever, nausea, vomiting, increased blood pressure, chills, musculoskeletal pain, increased heart rate, dyspnea, and reactions at the infusion sites.

Read the full prescribing information here.

The Food and Drug Administration has approved Octagam 10% as the first intravenous immunoglobulin with an indication specifically for adult dermatomyositis, according to a statement from manufacturer Octapharma USA.

Dermatomyositis is a rare, idiopathic autoimmune disorder that affects approximately 10 out of every million people in the United States, mainly adults in their late 40s to early 60s, according to the company, but children aged 5-15 years can be affected. The disease is characterized by skin rashes, chronic muscle inflammation, progressive muscle weakness, and risk for mortality that is three times higher than for the general population.

There are no previously approved treatments for dermatomyositis prior to Octagam 10%, which also is indicated for chronic immune thrombocytopenic purpura in adults.

The approval for dermatomyositis was based on the results of a phase 3 randomized, double-blind, placebo-controlled clinical trial (the ProDERM trial) that included 95 adult patients at 36 sites worldwide, with 17 sites in the United States. In the trial, 78.7% of patients with dermatomyositis who were randomized to receive 2 g/kg of Octagam 10% every 4 weeks showed response at 16 weeks, compared with 43.8% of patients who received placebo. Response was based on the 2016 American College of Rheumatology/European Alliance of Associations for Rheumatology myositis response criteria. Placebo patients who switched to intravenous immunoglobulin (IVIG) during a trial extension had response rates at week 40 similar to the original patients at week 16.

“The study gives clinicians much more confidence in the efficacy and safety of intravenous immunoglobulin and provides valuable information about what type of patient is best suited for the treatment,” Rohit Aggarwal, MD, medical director of the Arthritis and Autoimmunity Center at the University of Pittsburgh and a member of the ProDERM study Steering Committee, said in the Octapharma statement.

Safety and tolerability were similar to profiles seen with other IVIG medications, according to the statement. The medication does carry a boxed warning from its chronic ITP approval, cautioning about the potential for thrombosis, renal dysfunction, and acute renal failure.

The most common adverse reactions reported by dermatomyositis patients in the ProDERM trial were headache, fever, nausea, vomiting, increased blood pressure, chills, musculoskeletal pain, increased heart rate, dyspnea, and reactions at the infusion sites.

Read the full prescribing information here.

The Food and Drug Administration has approved Octagam 10% as the first intravenous immunoglobulin with an indication specifically for adult dermatomyositis, according to a statement from manufacturer Octapharma USA.

Dermatomyositis is a rare, idiopathic autoimmune disorder that affects approximately 10 out of every million people in the United States, mainly adults in their late 40s to early 60s, according to the company, but children aged 5-15 years can be affected. The disease is characterized by skin rashes, chronic muscle inflammation, progressive muscle weakness, and risk for mortality that is three times higher than for the general population.

There are no previously approved treatments for dermatomyositis prior to Octagam 10%, which also is indicated for chronic immune thrombocytopenic purpura in adults.

The approval for dermatomyositis was based on the results of a phase 3 randomized, double-blind, placebo-controlled clinical trial (the ProDERM trial) that included 95 adult patients at 36 sites worldwide, with 17 sites in the United States. In the trial, 78.7% of patients with dermatomyositis who were randomized to receive 2 g/kg of Octagam 10% every 4 weeks showed response at 16 weeks, compared with 43.8% of patients who received placebo. Response was based on the 2016 American College of Rheumatology/European Alliance of Associations for Rheumatology myositis response criteria. Placebo patients who switched to intravenous immunoglobulin (IVIG) during a trial extension had response rates at week 40 similar to the original patients at week 16.

“The study gives clinicians much more confidence in the efficacy and safety of intravenous immunoglobulin and provides valuable information about what type of patient is best suited for the treatment,” Rohit Aggarwal, MD, medical director of the Arthritis and Autoimmunity Center at the University of Pittsburgh and a member of the ProDERM study Steering Committee, said in the Octapharma statement.

Safety and tolerability were similar to profiles seen with other IVIG medications, according to the statement. The medication does carry a boxed warning from its chronic ITP approval, cautioning about the potential for thrombosis, renal dysfunction, and acute renal failure.

The most common adverse reactions reported by dermatomyositis patients in the ProDERM trial were headache, fever, nausea, vomiting, increased blood pressure, chills, musculoskeletal pain, increased heart rate, dyspnea, and reactions at the infusion sites.

Read the full prescribing information here.

A 6-year-old boy was seen in the hospital in consultation for a 3-week history of suspected cellulitis of the right ear. Drainage from the right ear was refractory to treatment with a 7-day course of cephalexin 15 mL po bid of 250 mg/5 mL solution and clindamycin 24.4 mL po tid of 75 mg/5 mL solution. Treatment was followed by admission to the hospital for treatment with intravenous (IV) cefazolin 1000 mg q6h and IV vancomycin 825 mg q6h for 1 week.

The patient had a significant past medical history for asthma, allergic rhinitis, and severe atopic dermatitis that had been treated with methotrexate 10 mg per week for 6 months beginning when the child was 5 years of age. When the methotrexate proved to be ineffective, the patient was started on Aquaphor and mometasone 0.1% ointment. A 6-month trial of these agents failed as well.

Physical examination revealed that the right ear and skin around it were edematous, erythematous, pruritic, and tender. There was also purulent drainage coming from the ear (FIGURE 1).

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

Diagnosis: Infectious eczematoid dermatitis

The patient was referred to a dermatologist after seeing an ear, nose, and throat (ENT) specialist who made the diagnosis of otitis externa when the rash failed to respond to topical and systemic antibiotics. The patient’s tender, pruritic, oozing, edematous eruption was recognized as an infectious eczematoid dermatitis (IED).

The ears, nose, and face are predominantly involved in cases of infectious eczematoid dermatitis in the pediatric population, while the lower extremities are predominantly involved in adults.

Although it is not an uncommon condition, IED may be underrecognized. It accounted for 2.9% of admissions to a ­dermatology-run inpatient service between 2000 and 2010.¹ IED results from cutaneous sensitization to purulent drainage secondary to acute otitis externa or another primary infection.² In fact, cultures from the purulent drainage in this patient grew methicillin-­resistant Staphylococcus aureus. The patient’s right otitis externa drainage may have been associated with the previous history of atopic dermatitis. Atopic dermatitis is associated with an increased risk of skin infections due to decreased inflammatory mediators (defensins).

Cellulitis and herpes zoster oticus are part of the differential

The differential diagnosis in this case includes bacterial cellulitis, acute otitis media, and herpes zoster oticus.

Bacterial cellulitis manifests with erythema, edema, and tenderness with blistering when associated with bullous impetigo rather than pruritus. The clinical appearance of the patient’s diffuse, weeping, edematous external ear, the lack of response to guided antibiotic therapy, and the pruritus experienced by the patient argue against the diagnosis of bacterial cellulitis.

Acute otitis media, like otitis externa, produces ear discharge usually associated with significant pain. Thus, it is important when working through the differential to define the source of the ear discharge. In this case, a consultation with an ENT specialist confirmed that there was an intact tympanic membrane with no middle ear involvement, ruling out the diagnosis of acute otitis media.

Continue to: Herpes zoster oticus

Herpes zoster oticus. The absence of grouped vesicles at any point during the eruption, itching rather than pain, and negative viral culture and polymerase chain reaction studies for herpes simplex and varicella zoster virus excluded the diagnosis of herpes zoster oticus.

Diagnostic criteria were met

This case was compatible with the characterizations of IED as initially described by Engman³ in 1902 and further detailed by Sutton,⁴ who provided the following criteria for diagnosis:

• an initial eczematous or pustular lesion
• extension peripherally by autoinoculation
• an absence of central clearing
• Staphylococcus on culture of the initial lesion
• a history of infection.

Case reports have added to our understanding of the mechanism of autosensitization of surrounding skin.⁵ Yamany and Schwartz have proposed the diagnostic criteria summarized in the TABLE.²

Age factors into location. The ears, nose, and face are predominantly involved in cases of IED in the pediatric population, while the lower extremities are predominantly involved in adults.⁶ Laboratory tests and imaging may aid in excluding other potential diagnoses or complications, but the diagnosis remains clinical and requires the clinician to avoid jumping to the conclusion that every moist, erythematous crusting eruption is purely infectious in nature.

Tx and prevention hinge on a combination of antibiotics, steroids

The management of IED should be aimed at fighting the infection, eliminating the allergic contact dermatitis associated with infectious products, and improving barrier protection. Topical and/or systemic antibiotics guided by culture focus on killing bacteria. The allergic immune response is dampened by systemic steroids. Topical steroids, however, are difficult to utilize on moist, draining skin. In the case of otitis externa, a combination topical antibiotic and steroid otic drop can be utilized. As healing begins, emollients are applied to aid in skin repair.² Topical antibiotics containing neomycin or polymyxin should be avoided to eliminate the possibility of developing contact sensitivity to these agents.

Topical antibiotics containing neomycin or polymyxin should be avoided to eliminate the possibility of developing contact sensitivity to these agents.

For our patient, inpatient wound cultures demonstrated methicillin-resistant S aureus, and empiric treatment with IV cefepime and vancomycin was transitioned to IV clindamycin based on sensitivities and then transitioned to a 12-day course of oral clindamycin 150 mg bid. In addition, the patient received ciprofloxacin/dexamethasone otic drops 3 times/d to treat his otitis externa. After initiating prednisone 30 mg (1 mg/kg/d) for 10 days to cover the allergic component, the patient showed prompt clinical improvement. Gentle cleansing of the right ear with hypoallergenic soap and water followed by application of petrolatum ointment 4 times/d was used to promote healing and improve barrier function (FIGURE 2). The patient’s mother indicated during a follow-up call that the affected area had dramatically improved.

A 6-year-old boy was seen in the hospital in consultation for a 3-week history of suspected cellulitis of the right ear. Drainage from the right ear was refractory to treatment with a 7-day course of cephalexin 15 mL po bid of 250 mg/5 mL solution and clindamycin 24.4 mL po tid of 75 mg/5 mL solution. Treatment was followed by admission to the hospital for treatment with intravenous (IV) cefazolin 1000 mg q6h and IV vancomycin 825 mg q6h for 1 week.

The patient had a significant past medical history for asthma, allergic rhinitis, and severe atopic dermatitis that had been treated with methotrexate 10 mg per week for 6 months beginning when the child was 5 years of age. When the methotrexate proved to be ineffective, the patient was started on Aquaphor and mometasone 0.1% ointment. A 6-month trial of these agents failed as well.

Physical examination revealed that the right ear and skin around it were edematous, erythematous, pruritic, and tender. There was also purulent drainage coming from the ear (FIGURE 1).

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

Diagnosis: Infectious eczematoid dermatitis

The patient was referred to a dermatologist after seeing an ear, nose, and throat (ENT) specialist who made the diagnosis of otitis externa when the rash failed to respond to topical and systemic antibiotics. The patient’s tender, pruritic, oozing, edematous eruption was recognized as an infectious eczematoid dermatitis (IED).

The ears, nose, and face are predominantly involved in cases of infectious eczematoid dermatitis in the pediatric population, while the lower extremities are predominantly involved in adults.

Although it is not an uncommon condition, IED may be underrecognized. It accounted for 2.9% of admissions to a ­dermatology-run inpatient service between 2000 and 2010.¹ IED results from cutaneous sensitization to purulent drainage secondary to acute otitis externa or another primary infection.² In fact, cultures from the purulent drainage in this patient grew methicillin-­resistant Staphylococcus aureus. The patient’s right otitis externa drainage may have been associated with the previous history of atopic dermatitis. Atopic dermatitis is associated with an increased risk of skin infections due to decreased inflammatory mediators (defensins).

Cellulitis and herpes zoster oticus are part of the differential

The differential diagnosis in this case includes bacterial cellulitis, acute otitis media, and herpes zoster oticus.

Bacterial cellulitis manifests with erythema, edema, and tenderness with blistering when associated with bullous impetigo rather than pruritus. The clinical appearance of the patient’s diffuse, weeping, edematous external ear, the lack of response to guided antibiotic therapy, and the pruritus experienced by the patient argue against the diagnosis of bacterial cellulitis.

Acute otitis media, like otitis externa, produces ear discharge usually associated with significant pain. Thus, it is important when working through the differential to define the source of the ear discharge. In this case, a consultation with an ENT specialist confirmed that there was an intact tympanic membrane with no middle ear involvement, ruling out the diagnosis of acute otitis media.

Continue to: Herpes zoster oticus

Herpes zoster oticus. The absence of grouped vesicles at any point during the eruption, itching rather than pain, and negative viral culture and polymerase chain reaction studies for herpes simplex and varicella zoster virus excluded the diagnosis of herpes zoster oticus.

Diagnostic criteria were met

This case was compatible with the characterizations of IED as initially described by Engman³ in 1902 and further detailed by Sutton,⁴ who provided the following criteria for diagnosis:

• an initial eczematous or pustular lesion
• extension peripherally by autoinoculation
• an absence of central clearing
• Staphylococcus on culture of the initial lesion
• a history of infection.

Case reports have added to our understanding of the mechanism of autosensitization of surrounding skin.⁵ Yamany and Schwartz have proposed the diagnostic criteria summarized in the TABLE.²

Age factors into location. The ears, nose, and face are predominantly involved in cases of IED in the pediatric population, while the lower extremities are predominantly involved in adults.⁶ Laboratory tests and imaging may aid in excluding other potential diagnoses or complications, but the diagnosis remains clinical and requires the clinician to avoid jumping to the conclusion that every moist, erythematous crusting eruption is purely infectious in nature.

Tx and prevention hinge on a combination of antibiotics, steroids

The management of IED should be aimed at fighting the infection, eliminating the allergic contact dermatitis associated with infectious products, and improving barrier protection. Topical and/or systemic antibiotics guided by culture focus on killing bacteria. The allergic immune response is dampened by systemic steroids. Topical steroids, however, are difficult to utilize on moist, draining skin. In the case of otitis externa, a combination topical antibiotic and steroid otic drop can be utilized. As healing begins, emollients are applied to aid in skin repair.² Topical antibiotics containing neomycin or polymyxin should be avoided to eliminate the possibility of developing contact sensitivity to these agents.

Topical antibiotics containing neomycin or polymyxin should be avoided to eliminate the possibility of developing contact sensitivity to these agents.

For our patient, inpatient wound cultures demonstrated methicillin-resistant S aureus, and empiric treatment with IV cefepime and vancomycin was transitioned to IV clindamycin based on sensitivities and then transitioned to a 12-day course of oral clindamycin 150 mg bid. In addition, the patient received ciprofloxacin/dexamethasone otic drops 3 times/d to treat his otitis externa. After initiating prednisone 30 mg (1 mg/kg/d) for 10 days to cover the allergic component, the patient showed prompt clinical improvement. Gentle cleansing of the right ear with hypoallergenic soap and water followed by application of petrolatum ointment 4 times/d was used to promote healing and improve barrier function (FIGURE 2). The patient’s mother indicated during a follow-up call that the affected area had dramatically improved.

A 6-year-old boy was seen in the hospital in consultation for a 3-week history of suspected cellulitis of the right ear. Drainage from the right ear was refractory to treatment with a 7-day course of cephalexin 15 mL po bid of 250 mg/5 mL solution and clindamycin 24.4 mL po tid of 75 mg/5 mL solution. Treatment was followed by admission to the hospital for treatment with intravenous (IV) cefazolin 1000 mg q6h and IV vancomycin 825 mg q6h for 1 week.

The patient had a significant past medical history for asthma, allergic rhinitis, and severe atopic dermatitis that had been treated with methotrexate 10 mg per week for 6 months beginning when the child was 5 years of age. When the methotrexate proved to be ineffective, the patient was started on Aquaphor and mometasone 0.1% ointment. A 6-month trial of these agents failed as well.

Physical examination revealed that the right ear and skin around it were edematous, erythematous, pruritic, and tender. There was also purulent drainage coming from the ear (FIGURE 1).

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

Diagnosis: Infectious eczematoid dermatitis

The patient was referred to a dermatologist after seeing an ear, nose, and throat (ENT) specialist who made the diagnosis of otitis externa when the rash failed to respond to topical and systemic antibiotics. The patient’s tender, pruritic, oozing, edematous eruption was recognized as an infectious eczematoid dermatitis (IED).

The ears, nose, and face are predominantly involved in cases of infectious eczematoid dermatitis in the pediatric population, while the lower extremities are predominantly involved in adults.

Although it is not an uncommon condition, IED may be underrecognized. It accounted for 2.9% of admissions to a ­dermatology-run inpatient service between 2000 and 2010.¹ IED results from cutaneous sensitization to purulent drainage secondary to acute otitis externa or another primary infection.² In fact, cultures from the purulent drainage in this patient grew methicillin-­resistant Staphylococcus aureus. The patient’s right otitis externa drainage may have been associated with the previous history of atopic dermatitis. Atopic dermatitis is associated with an increased risk of skin infections due to decreased inflammatory mediators (defensins).

Cellulitis and herpes zoster oticus are part of the differential

The differential diagnosis in this case includes bacterial cellulitis, acute otitis media, and herpes zoster oticus.

Bacterial cellulitis manifests with erythema, edema, and tenderness with blistering when associated with bullous impetigo rather than pruritus. The clinical appearance of the patient’s diffuse, weeping, edematous external ear, the lack of response to guided antibiotic therapy, and the pruritus experienced by the patient argue against the diagnosis of bacterial cellulitis.

Acute otitis media, like otitis externa, produces ear discharge usually associated with significant pain. Thus, it is important when working through the differential to define the source of the ear discharge. In this case, a consultation with an ENT specialist confirmed that there was an intact tympanic membrane with no middle ear involvement, ruling out the diagnosis of acute otitis media.

Continue to: Herpes zoster oticus

Herpes zoster oticus. The absence of grouped vesicles at any point during the eruption, itching rather than pain, and negative viral culture and polymerase chain reaction studies for herpes simplex and varicella zoster virus excluded the diagnosis of herpes zoster oticus.

Diagnostic criteria were met

This case was compatible with the characterizations of IED as initially described by Engman³ in 1902 and further detailed by Sutton,⁴ who provided the following criteria for diagnosis:

• an initial eczematous or pustular lesion
• extension peripherally by autoinoculation
• an absence of central clearing
• Staphylococcus on culture of the initial lesion
• a history of infection.

Case reports have added to our understanding of the mechanism of autosensitization of surrounding skin.⁵ Yamany and Schwartz have proposed the diagnostic criteria summarized in the TABLE.²

Age factors into location. The ears, nose, and face are predominantly involved in cases of IED in the pediatric population, while the lower extremities are predominantly involved in adults.⁶ Laboratory tests and imaging may aid in excluding other potential diagnoses or complications, but the diagnosis remains clinical and requires the clinician to avoid jumping to the conclusion that every moist, erythematous crusting eruption is purely infectious in nature.

Tx and prevention hinge on a combination of antibiotics, steroids

The management of IED should be aimed at fighting the infection, eliminating the allergic contact dermatitis associated with infectious products, and improving barrier protection. Topical and/or systemic antibiotics guided by culture focus on killing bacteria. The allergic immune response is dampened by systemic steroids. Topical steroids, however, are difficult to utilize on moist, draining skin. In the case of otitis externa, a combination topical antibiotic and steroid otic drop can be utilized. As healing begins, emollients are applied to aid in skin repair.² Topical antibiotics containing neomycin or polymyxin should be avoided to eliminate the possibility of developing contact sensitivity to these agents.

Topical antibiotics containing neomycin or polymyxin should be avoided to eliminate the possibility of developing contact sensitivity to these agents.

For our patient, inpatient wound cultures demonstrated methicillin-resistant S aureus, and empiric treatment with IV cefepime and vancomycin was transitioned to IV clindamycin based on sensitivities and then transitioned to a 12-day course of oral clindamycin 150 mg bid. In addition, the patient received ciprofloxacin/dexamethasone otic drops 3 times/d to treat his otitis externa. After initiating prednisone 30 mg (1 mg/kg/d) for 10 days to cover the allergic component, the patient showed prompt clinical improvement. Gentle cleansing of the right ear with hypoallergenic soap and water followed by application of petrolatum ointment 4 times/d was used to promote healing and improve barrier function (FIGURE 2). The patient’s mother indicated during a follow-up call that the affected area had dramatically improved.

Declining trends in melanoma death rates accelerated from 2014 to 2018 in the United States, even as incidence rates increased for both males and females, according to an annual report by several national organizations.

“Death rates for cutaneous melanoma have declined rapidly in recent years following introduction of new therapies, including targeted and immune checkpoint inhibitors, the first of which was approved by the [Food and Drug Administration] in early 2011,” Farhad Islami, MD, PhD, of the American Cancer Society, and associates wrote in the Journal of the National Cancer Institute.

The American Cancer Society, along with the Centers for Disease Control and Prevention, the National Cancer Institute, and the North American Association of Central Cancer Registries, issue a joint report each year to update the incidence and mortality of the most common cancers and analyze short- and long-term trends since 2001.

Long-term melanoma mortality gets divided into two trends: First a slow decline over about a decade, then an accelerated decline until the end of the study period, although the timing is slightly different between males and females. For men, the death rate fell by an average of 0.9% a year from 2001 to 2009, compared with 5.7% per year in 2013-2018. For women, the average annual change went from –0.3% for 2001-2012 to –4.4% in 2012-2018.

The incidence of melanoma, however, headed in the opposite direction, rising 1.9% per year for females and 2.2% for males from 2001 to 2017, without the notable change in trend seen with death rates, Dr. Islami and associates said.

Incidence by race/ethnicity, reported for 2013-2017, shows that melanoma is much more common among white non-Hispanics: 37.4 per 100,000 standard population for males and 24.5 for females. Non-Hispanic American Indians/Alaska Natives were next at 10.8 (men) and 6.7 (women), followed by Hispanics (5.1/4.5), non-Hispanic Asians/Pacific Islanders (1.6/1.3), and non-Hispanic Blacks (1.2/1.0), they reported.

Death rates for melanoma, reported for 2014-2018, follow a similar pattern. White males (4.2 per 100,000) and females (1.8 per 100,000) had the highest mortality, then American Indians/Alaska Natives (1.0/0.5) and Hispanics (0.9/0.5), but rates were the same for Blacks and Asians/Pacific Islanders (0.4/0.3), the investigators said.

The accelerated decline in death rates in more recent years reflects “a substantial increase in survival for metastatic melanoma,” the participating organizations noted in a joint statement.

Increases in 2-year survival in distant-stage disease averaged 3.1% per year for those diagnosed during 2009-2014, which “slightly preceded the FDA approval of new therapies, likely because of the administration of these therapies through clinical trials and the FDA expanded access programs prior to the approval,” Dr. Islami and associates wrote.

The 2-year relative survival for those with nonmetastatic melanoma also improved over the study period, but the increases were much smaller: 0.4% per year for regional-stage disease and just 0.03% localized-stage cases diagnosed in 2001-2014, they reported.

The report was funded by the four participating groups. Six of the 12 investigators are employees of the American Cancer Society whose salaries are solely paid by the society; the other authors had no conflicts of interest to disclose.

rfranki@mdedge.com

Declining trends in melanoma death rates accelerated from 2014 to 2018 in the United States, even as incidence rates increased for both males and females, according to an annual report by several national organizations.

“Death rates for cutaneous melanoma have declined rapidly in recent years following introduction of new therapies, including targeted and immune checkpoint inhibitors, the first of which was approved by the [Food and Drug Administration] in early 2011,” Farhad Islami, MD, PhD, of the American Cancer Society, and associates wrote in the Journal of the National Cancer Institute.

The American Cancer Society, along with the Centers for Disease Control and Prevention, the National Cancer Institute, and the North American Association of Central Cancer Registries, issue a joint report each year to update the incidence and mortality of the most common cancers and analyze short- and long-term trends since 2001.

Long-term melanoma mortality gets divided into two trends: First a slow decline over about a decade, then an accelerated decline until the end of the study period, although the timing is slightly different between males and females. For men, the death rate fell by an average of 0.9% a year from 2001 to 2009, compared with 5.7% per year in 2013-2018. For women, the average annual change went from –0.3% for 2001-2012 to –4.4% in 2012-2018.

The incidence of melanoma, however, headed in the opposite direction, rising 1.9% per year for females and 2.2% for males from 2001 to 2017, without the notable change in trend seen with death rates, Dr. Islami and associates said.

Incidence by race/ethnicity, reported for 2013-2017, shows that melanoma is much more common among white non-Hispanics: 37.4 per 100,000 standard population for males and 24.5 for females. Non-Hispanic American Indians/Alaska Natives were next at 10.8 (men) and 6.7 (women), followed by Hispanics (5.1/4.5), non-Hispanic Asians/Pacific Islanders (1.6/1.3), and non-Hispanic Blacks (1.2/1.0), they reported.

Death rates for melanoma, reported for 2014-2018, follow a similar pattern. White males (4.2 per 100,000) and females (1.8 per 100,000) had the highest mortality, then American Indians/Alaska Natives (1.0/0.5) and Hispanics (0.9/0.5), but rates were the same for Blacks and Asians/Pacific Islanders (0.4/0.3), the investigators said.

The accelerated decline in death rates in more recent years reflects “a substantial increase in survival for metastatic melanoma,” the participating organizations noted in a joint statement.

Increases in 2-year survival in distant-stage disease averaged 3.1% per year for those diagnosed during 2009-2014, which “slightly preceded the FDA approval of new therapies, likely because of the administration of these therapies through clinical trials and the FDA expanded access programs prior to the approval,” Dr. Islami and associates wrote.

The 2-year relative survival for those with nonmetastatic melanoma also improved over the study period, but the increases were much smaller: 0.4% per year for regional-stage disease and just 0.03% localized-stage cases diagnosed in 2001-2014, they reported.

The report was funded by the four participating groups. Six of the 12 investigators are employees of the American Cancer Society whose salaries are solely paid by the society; the other authors had no conflicts of interest to disclose.

rfranki@mdedge.com

Declining trends in melanoma death rates accelerated from 2014 to 2018 in the United States, even as incidence rates increased for both males and females, according to an annual report by several national organizations.

“Death rates for cutaneous melanoma have declined rapidly in recent years following introduction of new therapies, including targeted and immune checkpoint inhibitors, the first of which was approved by the [Food and Drug Administration] in early 2011,” Farhad Islami, MD, PhD, of the American Cancer Society, and associates wrote in the Journal of the National Cancer Institute.

The American Cancer Society, along with the Centers for Disease Control and Prevention, the National Cancer Institute, and the North American Association of Central Cancer Registries, issue a joint report each year to update the incidence and mortality of the most common cancers and analyze short- and long-term trends since 2001.

Long-term melanoma mortality gets divided into two trends: First a slow decline over about a decade, then an accelerated decline until the end of the study period, although the timing is slightly different between males and females. For men, the death rate fell by an average of 0.9% a year from 2001 to 2009, compared with 5.7% per year in 2013-2018. For women, the average annual change went from –0.3% for 2001-2012 to –4.4% in 2012-2018.

The incidence of melanoma, however, headed in the opposite direction, rising 1.9% per year for females and 2.2% for males from 2001 to 2017, without the notable change in trend seen with death rates, Dr. Islami and associates said.

Incidence by race/ethnicity, reported for 2013-2017, shows that melanoma is much more common among white non-Hispanics: 37.4 per 100,000 standard population for males and 24.5 for females. Non-Hispanic American Indians/Alaska Natives were next at 10.8 (men) and 6.7 (women), followed by Hispanics (5.1/4.5), non-Hispanic Asians/Pacific Islanders (1.6/1.3), and non-Hispanic Blacks (1.2/1.0), they reported.

Death rates for melanoma, reported for 2014-2018, follow a similar pattern. White males (4.2 per 100,000) and females (1.8 per 100,000) had the highest mortality, then American Indians/Alaska Natives (1.0/0.5) and Hispanics (0.9/0.5), but rates were the same for Blacks and Asians/Pacific Islanders (0.4/0.3), the investigators said.

The accelerated decline in death rates in more recent years reflects “a substantial increase in survival for metastatic melanoma,” the participating organizations noted in a joint statement.

Increases in 2-year survival in distant-stage disease averaged 3.1% per year for those diagnosed during 2009-2014, which “slightly preceded the FDA approval of new therapies, likely because of the administration of these therapies through clinical trials and the FDA expanded access programs prior to the approval,” Dr. Islami and associates wrote.

The 2-year relative survival for those with nonmetastatic melanoma also improved over the study period, but the increases were much smaller: 0.4% per year for regional-stage disease and just 0.03% localized-stage cases diagnosed in 2001-2014, they reported.

The report was funded by the four participating groups. Six of the 12 investigators are employees of the American Cancer Society whose salaries are solely paid by the society; the other authors had no conflicts of interest to disclose.

rfranki@mdedge.com

ANSWER

The correct answer is all of the above (choice “f”).

DISCUSSION

The most likely diagnostic explanation for this patient’s presentation is erythema annulare centrifugum (EAC; choice “c”). However, this diagnosis is often difficult to establish, in part because of the broad differential and also because the very existence of EAC is far from well established.

The overwhelming consensus is that EAC represents a hypersensitivity reaction to an unknown antigen. It can be triggered by a wide variety of micro-organisms, stress, or even pregnancy.

In this case, there was no clinical or historical reason to suspect an underlying cancer, Lyme disease, or lupus, nor did the biopsy results suggest any of these.

The key takeaway here is to urge providers to avoid jumping onto a diagnostic bandwagon before considering a wider differential. Indeed, size matters when it relates to the length of one’s differential diagnosis list. If you don’t consider it, you can’t diagnose it.

TREATMENT

Fortunately, EAC nearly always resolves, with or without treatment. This patient received reassurance as such but was scheduled to return for a check of his lesions in 2 months. At that point, they had resolved.

ANSWER

The correct answer is all of the above (choice “f”).

DISCUSSION

The most likely diagnostic explanation for this patient’s presentation is erythema annulare centrifugum (EAC; choice “c”). However, this diagnosis is often difficult to establish, in part because of the broad differential and also because the very existence of EAC is far from well established.

The overwhelming consensus is that EAC represents a hypersensitivity reaction to an unknown antigen. It can be triggered by a wide variety of micro-organisms, stress, or even pregnancy.

In this case, there was no clinical or historical reason to suspect an underlying cancer, Lyme disease, or lupus, nor did the biopsy results suggest any of these.

The key takeaway here is to urge providers to avoid jumping onto a diagnostic bandwagon before considering a wider differential. Indeed, size matters when it relates to the length of one’s differential diagnosis list. If you don’t consider it, you can’t diagnose it.

TREATMENT

Fortunately, EAC nearly always resolves, with or without treatment. This patient received reassurance as such but was scheduled to return for a check of his lesions in 2 months. At that point, they had resolved.

ANSWER

The correct answer is all of the above (choice “f”).

DISCUSSION

The most likely diagnostic explanation for this patient’s presentation is erythema annulare centrifugum (EAC; choice “c”). However, this diagnosis is often difficult to establish, in part because of the broad differential and also because the very existence of EAC is far from well established.

The overwhelming consensus is that EAC represents a hypersensitivity reaction to an unknown antigen. It can be triggered by a wide variety of micro-organisms, stress, or even pregnancy.

In this case, there was no clinical or historical reason to suspect an underlying cancer, Lyme disease, or lupus, nor did the biopsy results suggest any of these.

The key takeaway here is to urge providers to avoid jumping onto a diagnostic bandwagon before considering a wider differential. Indeed, size matters when it relates to the length of one’s differential diagnosis list. If you don’t consider it, you can’t diagnose it.

TREATMENT

Fortunately, EAC nearly always resolves, with or without treatment. This patient received reassurance as such but was scheduled to return for a check of his lesions in 2 months. At that point, they had resolved.

Trypanosomatidae

Leishmaniasis is caused by protozoa of the family Trypanosomatidae, called Leishmania. The vector is a sandfly infected with the protozoa.¹

The three main forms of leishmaniasis – cutaneous, mucocutaneous, or visceral – varies with the species of organism involved, the geographic distribution, and the immune response of the patient. A majority of the cases seen in the United States are from patients who contracted the disease elsewhere, particularly from Peru and Brazil.²Cutaneous leishmaniasis (CL) is categorized as Old World (Baghdad boil, leishmaniasis tropica) or New World (uta, pian bois, bay sore, chiclero ulcer).^3,4

Lesions can vary from asymptomatic to severe. The initial lesion typically develops within weeks or months, and presents as an erythematous papule that is seen at the bite site.³ The papule evolves into a nodule or plaque that may ulcerate and crust.³ The ulcer can be distinguished by a raised and distinct border. In older stages, atrophic scarring may be seen. In some cases, the lesions may present years after exposure, because of immunosuppression or trauma.

Histology of CL reveals tuberculoid granulomas with parasitized histiocytes present. Amastigotes with distinct nuclei and kinetoplasts characterize Leishmania.2 In addition to histology, the biopsy may be sent for the press-imprint-smear method (PIS). In a study of 75 patients, the PIS method showed a higher sensitivity, as well as being a less costly and more rapid option for diagnosis.⁵

The treatment depends on the severity of the lesion and the species of the Leishmania genus. Mild lesions may resolve spontaneously. Topical imiquimod, cryotherapy, photodynamic therapy, and heat therapy may aid in the healing process.⁵ Systemic azole antifungal medications, miltefosine, and amphotericin B, and pentamidine may be used for more persistent lesions. In very severe cases, pentavalent antimonials (sodium stibogluconate, Pentostam) may be administered intravenously, although there is a high occurrence of recorded side effects.²

This case and the photos were submitted by Sabrina Liao, BS, University of California, San Diego; and Brooke Resh Sateesh, MD, San Diego Family Dermatology The case was edited by Donna Bilu Martin, MD.
 

References

1. Leishmaniasis – Resources for Health Professionals. Centers for Disease Control and Prevention. 2021 Jun 3.

2. Stark CG. Leishmaniasis. Medscape. 2020 Feb 18.

3. Markle WH and Makhoul K. Am Fam Physician. 2004 Mar 15;69(6):1455-604.

4. Ngan V. Leishmaniasis. DermNet NZ. 2017 Jan. 7.

5. Sousa AQ et al. Am J Trop Med Hyg. 2014 Nov;91(5):905-7.
 

Trypanosomatidae

Leishmaniasis is caused by protozoa of the family Trypanosomatidae, called Leishmania. The vector is a sandfly infected with the protozoa.¹

The three main forms of leishmaniasis – cutaneous, mucocutaneous, or visceral – varies with the species of organism involved, the geographic distribution, and the immune response of the patient. A majority of the cases seen in the United States are from patients who contracted the disease elsewhere, particularly from Peru and Brazil.²Cutaneous leishmaniasis (CL) is categorized as Old World (Baghdad boil, leishmaniasis tropica) or New World (uta, pian bois, bay sore, chiclero ulcer).^3,4

Lesions can vary from asymptomatic to severe. The initial lesion typically develops within weeks or months, and presents as an erythematous papule that is seen at the bite site.³ The papule evolves into a nodule or plaque that may ulcerate and crust.³ The ulcer can be distinguished by a raised and distinct border. In older stages, atrophic scarring may be seen. In some cases, the lesions may present years after exposure, because of immunosuppression or trauma.

Histology of CL reveals tuberculoid granulomas with parasitized histiocytes present. Amastigotes with distinct nuclei and kinetoplasts characterize Leishmania.2 In addition to histology, the biopsy may be sent for the press-imprint-smear method (PIS). In a study of 75 patients, the PIS method showed a higher sensitivity, as well as being a less costly and more rapid option for diagnosis.⁵

The treatment depends on the severity of the lesion and the species of the Leishmania genus. Mild lesions may resolve spontaneously. Topical imiquimod, cryotherapy, photodynamic therapy, and heat therapy may aid in the healing process.⁵ Systemic azole antifungal medications, miltefosine, and amphotericin B, and pentamidine may be used for more persistent lesions. In very severe cases, pentavalent antimonials (sodium stibogluconate, Pentostam) may be administered intravenously, although there is a high occurrence of recorded side effects.²

This case and the photos were submitted by Sabrina Liao, BS, University of California, San Diego; and Brooke Resh Sateesh, MD, San Diego Family Dermatology The case was edited by Donna Bilu Martin, MD.
 

References

1. Leishmaniasis – Resources for Health Professionals. Centers for Disease Control and Prevention. 2021 Jun 3.

2. Stark CG. Leishmaniasis. Medscape. 2020 Feb 18.

3. Markle WH and Makhoul K. Am Fam Physician. 2004 Mar 15;69(6):1455-604.

4. Ngan V. Leishmaniasis. DermNet NZ. 2017 Jan. 7.

5. Sousa AQ et al. Am J Trop Med Hyg. 2014 Nov;91(5):905-7.
 

Trypanosomatidae

Leishmaniasis is caused by protozoa of the family Trypanosomatidae, called Leishmania. The vector is a sandfly infected with the protozoa.¹

The three main forms of leishmaniasis – cutaneous, mucocutaneous, or visceral – varies with the species of organism involved, the geographic distribution, and the immune response of the patient. A majority of the cases seen in the United States are from patients who contracted the disease elsewhere, particularly from Peru and Brazil.²Cutaneous leishmaniasis (CL) is categorized as Old World (Baghdad boil, leishmaniasis tropica) or New World (uta, pian bois, bay sore, chiclero ulcer).^3,4

Lesions can vary from asymptomatic to severe. The initial lesion typically develops within weeks or months, and presents as an erythematous papule that is seen at the bite site.³ The papule evolves into a nodule or plaque that may ulcerate and crust.³ The ulcer can be distinguished by a raised and distinct border. In older stages, atrophic scarring may be seen. In some cases, the lesions may present years after exposure, because of immunosuppression or trauma.

Histology of CL reveals tuberculoid granulomas with parasitized histiocytes present. Amastigotes with distinct nuclei and kinetoplasts characterize Leishmania.2 In addition to histology, the biopsy may be sent for the press-imprint-smear method (PIS). In a study of 75 patients, the PIS method showed a higher sensitivity, as well as being a less costly and more rapid option for diagnosis.⁵

The treatment depends on the severity of the lesion and the species of the Leishmania genus. Mild lesions may resolve spontaneously. Topical imiquimod, cryotherapy, photodynamic therapy, and heat therapy may aid in the healing process.⁵ Systemic azole antifungal medications, miltefosine, and amphotericin B, and pentamidine may be used for more persistent lesions. In very severe cases, pentavalent antimonials (sodium stibogluconate, Pentostam) may be administered intravenously, although there is a high occurrence of recorded side effects.²

This case and the photos were submitted by Sabrina Liao, BS, University of California, San Diego; and Brooke Resh Sateesh, MD, San Diego Family Dermatology The case was edited by Donna Bilu Martin, MD.
 

References

1. Leishmaniasis – Resources for Health Professionals. Centers for Disease Control and Prevention. 2021 Jun 3.

2. Stark CG. Leishmaniasis. Medscape. 2020 Feb 18.

3. Markle WH and Makhoul K. Am Fam Physician. 2004 Mar 15;69(6):1455-604.

4. Ngan V. Leishmaniasis. DermNet NZ. 2017 Jan. 7.

5. Sousa AQ et al. Am J Trop Med Hyg. 2014 Nov;91(5):905-7.
 

Consumers should stop using certain brands of spray-on sunscreen products made by Johnson & Johnson. The company has issued a voluntary recall after finding low levels of benzene, a known cancer-causing agent, in some samples.

Benzene is not an ingredient of sunscreen, and should not be present in these products. The levels detected were low and would not be expected to have an adverse effect on health, but the company says it is recalling the products anyway “out of an abundance of caution.”

The sunscreen products that have been recalled are:

• NEUTROGENA® Beach Defense® aerosol sunscreen.
• NEUTROGENA® Cool Dry Sport aerosol sunscreen.
• NEUTROGENA® Invisible Daily™ defense aerosol sunscreen.
• NEUTROGENA® Ultra Sheer® aerosol sunscreen.
• AVEENO® Protect + Refresh aerosol sunscreen.

These products were distributed nationwide through a variety of retail stores. Consumers should stop using these products and throw them away, the company said.

At the same time, it emphasized the importance of using alternative sunscreen products to protect the skin from excessive sun exposure, which can lead to skin cancer including melanoma.

Johnson & Johnson has launched an investigation into how benzene got into these products.

One of the company’s other spray sunscreen products, Neutrogena Wet Skin, was not included in the recall.

Recently, benzene was found in 78 widely-used sunscreen products in tests conducted by the online pharmacy and laboratory Valisure. Most of the products were aerosol sprays, and the company called on the Food and Drug Administration to recall them all.

That petition suggested that the finding of benzene was the result of contamination somewhere in the manufacturing process.

“This isn’t a sunscreen issue, it’s a manufacturing issue,” said Adam Friedman, MD, professor and chief of dermatology at George Washington University. “We don’t want those things to be blurred.”

There is a risk that people take away the wrong message from these findings.

“People already have ambivalence about sunscreen, and this is just going to make that worse,” Dr. Friedman said in an interview.

He pointed out that benzene is present in car exhaust, second-hand smoke, and elsewhere. Inhalation exposure has been the primary focus of toxicology investigations, as has exposure from things such as contaminated drinking water – not via topical application. “We don’t know how effectively [benzene] gets through the skin, if it gets absorbed systemically, and how that then behaves downstream,” he noted.

On the other hand, ultraviolet radiation is a well-established carcinogen. Avoiding an effective preventive measure such as sunscreen could prove more harmful than exposure to trace amounts of benzene, he said.

A version of this article first appeared on WebMD.com.

Consumers should stop using certain brands of spray-on sunscreen products made by Johnson & Johnson. The company has issued a voluntary recall after finding low levels of benzene, a known cancer-causing agent, in some samples.

Benzene is not an ingredient of sunscreen, and should not be present in these products. The levels detected were low and would not be expected to have an adverse effect on health, but the company says it is recalling the products anyway “out of an abundance of caution.”

The sunscreen products that have been recalled are:

• NEUTROGENA® Beach Defense® aerosol sunscreen.
• NEUTROGENA® Cool Dry Sport aerosol sunscreen.
• NEUTROGENA® Invisible Daily™ defense aerosol sunscreen.
• NEUTROGENA® Ultra Sheer® aerosol sunscreen.
• AVEENO® Protect + Refresh aerosol sunscreen.

These products were distributed nationwide through a variety of retail stores. Consumers should stop using these products and throw them away, the company said.

At the same time, it emphasized the importance of using alternative sunscreen products to protect the skin from excessive sun exposure, which can lead to skin cancer including melanoma.

Johnson & Johnson has launched an investigation into how benzene got into these products.

One of the company’s other spray sunscreen products, Neutrogena Wet Skin, was not included in the recall.

Recently, benzene was found in 78 widely-used sunscreen products in tests conducted by the online pharmacy and laboratory Valisure. Most of the products were aerosol sprays, and the company called on the Food and Drug Administration to recall them all.

That petition suggested that the finding of benzene was the result of contamination somewhere in the manufacturing process.

“This isn’t a sunscreen issue, it’s a manufacturing issue,” said Adam Friedman, MD, professor and chief of dermatology at George Washington University. “We don’t want those things to be blurred.”

There is a risk that people take away the wrong message from these findings.

“People already have ambivalence about sunscreen, and this is just going to make that worse,” Dr. Friedman said in an interview.

He pointed out that benzene is present in car exhaust, second-hand smoke, and elsewhere. Inhalation exposure has been the primary focus of toxicology investigations, as has exposure from things such as contaminated drinking water – not via topical application. “We don’t know how effectively [benzene] gets through the skin, if it gets absorbed systemically, and how that then behaves downstream,” he noted.

On the other hand, ultraviolet radiation is a well-established carcinogen. Avoiding an effective preventive measure such as sunscreen could prove more harmful than exposure to trace amounts of benzene, he said.

A version of this article first appeared on WebMD.com.

Consumers should stop using certain brands of spray-on sunscreen products made by Johnson & Johnson. The company has issued a voluntary recall after finding low levels of benzene, a known cancer-causing agent, in some samples.

Benzene is not an ingredient of sunscreen, and should not be present in these products. The levels detected were low and would not be expected to have an adverse effect on health, but the company says it is recalling the products anyway “out of an abundance of caution.”

The sunscreen products that have been recalled are:

• NEUTROGENA® Beach Defense® aerosol sunscreen.
• NEUTROGENA® Cool Dry Sport aerosol sunscreen.
• NEUTROGENA® Invisible Daily™ defense aerosol sunscreen.
• NEUTROGENA® Ultra Sheer® aerosol sunscreen.
• AVEENO® Protect + Refresh aerosol sunscreen.

These products were distributed nationwide through a variety of retail stores. Consumers should stop using these products and throw them away, the company said.

At the same time, it emphasized the importance of using alternative sunscreen products to protect the skin from excessive sun exposure, which can lead to skin cancer including melanoma.

Johnson & Johnson has launched an investigation into how benzene got into these products.

One of the company’s other spray sunscreen products, Neutrogena Wet Skin, was not included in the recall.

Recently, benzene was found in 78 widely-used sunscreen products in tests conducted by the online pharmacy and laboratory Valisure. Most of the products were aerosol sprays, and the company called on the Food and Drug Administration to recall them all.

That petition suggested that the finding of benzene was the result of contamination somewhere in the manufacturing process.

“This isn’t a sunscreen issue, it’s a manufacturing issue,” said Adam Friedman, MD, professor and chief of dermatology at George Washington University. “We don’t want those things to be blurred.”

There is a risk that people take away the wrong message from these findings.

“People already have ambivalence about sunscreen, and this is just going to make that worse,” Dr. Friedman said in an interview.

He pointed out that benzene is present in car exhaust, second-hand smoke, and elsewhere. Inhalation exposure has been the primary focus of toxicology investigations, as has exposure from things such as contaminated drinking water – not via topical application. “We don’t know how effectively [benzene] gets through the skin, if it gets absorbed systemically, and how that then behaves downstream,” he noted.

On the other hand, ultraviolet radiation is a well-established carcinogen. Avoiding an effective preventive measure such as sunscreen could prove more harmful than exposure to trace amounts of benzene, he said.

A version of this article first appeared on WebMD.com.

The prevalence of pediatric alopecia areata (AA) in the United States has increased twofold over the past decade and it disproportionately affects females and Hispanic children, according to results from the largest study to date on the topic.

“Alopecia areata is a relatively common cause of nonscarring hair loss in children,” Paige McKenzie said during the annual meeting of the Society for Pediatric Dermatology. “The only two epidemiologic studies that have been performed in children have been based on registry or survey data which is inherently at risk for bias,” she added, referring to studies published in 2017 and 2018. “Additionally, epidemiologic descriptions of alopecia areata in adults are limited and overall estimates have varied from 0.2% to 2%. Current understanding is also largely based on population studies in Olmsted County, Minnesota, an area with mostly White racial demographics, so it’s not representative of the U.S. population as a whole.”

To identify the incidence and prevalence of pediatric AA over time, and across age, race/ethnicity, and sex, Ms. McKenzie and colleagues conducted a retrospective cohort study from 2009 to 2020 using PEDSnet, a network of seven U.S. pediatric health institutions with a database of more than 6.5 million children. “PEDSnet is unique because it uses a common data model to standardize EHR data across different health systems and uses SNOMED [Systematized Nomenclature of Medicine]–Clinical Terms to identify specific patient populations,” said Ms. McKenzie, who was a clinical research fellow in the section of dermatology at the Children’s Hospital of Philadelphia during the 2020-2021 academic year.

She and her coauthors limited their analysis to children younger than age 18 who were assigned a SNOMED code for AA during at least one dermatology physician visit or at least two nondermatology physician visits. They also identified an incidence cohort that was a subset of the study cohort who had at least 12 months of follow-up. “To determine the accuracy of AA patient identification, we also reviewed 100 cases at random from one institution with a threshold of greater than 95% accuracy,” said Ms. McKenzie, who is now a fourth-year medical student at the University of Texas Southwestern Medical Center, Dallas.

Of 5,409,919 children included in the study, 5,801 had AA, for an overall prevalence of 0.11%. The prevalence doubled from 0.04% in 2009 to 0.08% in 2019. “It fell in 2020, which we believe is a result of the COVID-19 pandemic’s effects on health care utilization,” she said. AA prevalence peaked at 9 years of age and was higher among females, compared with males (0.12% vs. 0.09%, respectively). The prevalence was highest among Hispanic children (0.23%), followed by Asian children (0.17%), Black children (0.12%), and White children (0.08%).

The incidence cohort consisted of 2,896,241 children. Of these, 2,398 had AA between 2009-2020, for an overall incidence of 13.6 cases per 100,000 patient-years. The incidence rate of AA by age was normally distributed and peaked at 6 years of age. Rates were 22.8% higher in female patients than in male patients. In addition, incidence rates were highest among Hispanics (31.5/100,000 person-years), followed by Asians (23.1/100,000 person-years), Blacks (17.0/100,000 person-years), and Whites (8.8/100,000).

Logistic regression analysis showed general agreement with the unadjusted incidence data. Males were less likely to be diagnosed with AA, compared with females (adjusted odds ratio, 0.80; P < .001). Analysis across race/ethnicity revealed significantly increased rates among children from minority backgrounds when compared with white children. Hispanic children had the greatest risk of developing AA (aOR, 3.07), followed by Asian children (aOR, 2.02), and Black children (aOR, 1.73) (P < .001 for all associations). Patients with atopic dermatitis, thyroid disease, psoriasis, vitiligo, and trisomy 21 prior to AA diagnosis all had a significantly higher risk of developing AA, compared with those without those diagnoses.

“This is the largest description of pediatric AA to date,” Ms. McKenzie said. “The prevalence has increased steadily, with a twofold increase over the last 10 years, which mirrors other autoimmune disorders. Children who identify as Hispanic, Asian, and Black have significantly higher incidence rates of alopecia areata compared to those who identify as White.”

Moving forward, she added, “efforts should focus on increasing education and awareness of AA in diverse communities and in community pediatricians so that patients can be diagnosed correctly early on. We can also use this data to ensure that representative populations are included in clinical trials for patients with AA.”

Asked to comment on the results Maria Hordinsky, MD, professor and chair of the department of dermatology at the University of Minnesota, Minneapolis, said that the study “is a great contribution to our understanding of the epidemiology of pediatric alopecia areata and also highlights how common alopecia areata is in children.” In an interview, she said that it would be interesting to see if this is a worldwide phenomenon or unique to the United States.

Lawrence J. Green, MD, clinical professor of dermatology at George Washington University, Washington, who was asked to comment on the study, characterized the work as being “very informative. Looking at a large cohort of pediatric patients with alopecia areata diagnosed by a dermatologist or two or more nondermatologists, the authors found a higher incidence and prevalence in nonwhite children here in the United States. I am worried in fact, the true incidence could be even higher than noted in the searched database because nonwhite children can often come from underserved and undercared for areas.”

The other authors were Christopher B. Forrest, MD, PhD, Mitchell Maltenfort, PhD, and Leslie Castelo-Soccio, MD, PhD, of Children’s Hospital of Philadelphia. Dr. Castelo-Soccio is a consultant for Pfizer; the other authors reported having no financial disclosures. Dr. Hordinsky disclosed receiving grant support for clinical research work on hair diseases from Pfizer, Eli Lilly, Concert Pharmaceuticals, and Target Derm and grant support from the National Alopecia Areata Foundation; and is on an advisory panel for Cassiopea. Dr. Green disclosed that he is a speaker, consultant, or investigator for numerous pharmaceutical companies.

*This story was updated on 7/19/21.

dbrunk@mdedge.com

The prevalence of pediatric alopecia areata (AA) in the United States has increased twofold over the past decade and it disproportionately affects females and Hispanic children, according to results from the largest study to date on the topic.

“Alopecia areata is a relatively common cause of nonscarring hair loss in children,” Paige McKenzie said during the annual meeting of the Society for Pediatric Dermatology. “The only two epidemiologic studies that have been performed in children have been based on registry or survey data which is inherently at risk for bias,” she added, referring to studies published in 2017 and 2018. “Additionally, epidemiologic descriptions of alopecia areata in adults are limited and overall estimates have varied from 0.2% to 2%. Current understanding is also largely based on population studies in Olmsted County, Minnesota, an area with mostly White racial demographics, so it’s not representative of the U.S. population as a whole.”

To identify the incidence and prevalence of pediatric AA over time, and across age, race/ethnicity, and sex, Ms. McKenzie and colleagues conducted a retrospective cohort study from 2009 to 2020 using PEDSnet, a network of seven U.S. pediatric health institutions with a database of more than 6.5 million children. “PEDSnet is unique because it uses a common data model to standardize EHR data across different health systems and uses SNOMED [Systematized Nomenclature of Medicine]–Clinical Terms to identify specific patient populations,” said Ms. McKenzie, who was a clinical research fellow in the section of dermatology at the Children’s Hospital of Philadelphia during the 2020-2021 academic year.

She and her coauthors limited their analysis to children younger than age 18 who were assigned a SNOMED code for AA during at least one dermatology physician visit or at least two nondermatology physician visits. They also identified an incidence cohort that was a subset of the study cohort who had at least 12 months of follow-up. “To determine the accuracy of AA patient identification, we also reviewed 100 cases at random from one institution with a threshold of greater than 95% accuracy,” said Ms. McKenzie, who is now a fourth-year medical student at the University of Texas Southwestern Medical Center, Dallas.

Of 5,409,919 children included in the study, 5,801 had AA, for an overall prevalence of 0.11%. The prevalence doubled from 0.04% in 2009 to 0.08% in 2019. “It fell in 2020, which we believe is a result of the COVID-19 pandemic’s effects on health care utilization,” she said. AA prevalence peaked at 9 years of age and was higher among females, compared with males (0.12% vs. 0.09%, respectively). The prevalence was highest among Hispanic children (0.23%), followed by Asian children (0.17%), Black children (0.12%), and White children (0.08%).

The incidence cohort consisted of 2,896,241 children. Of these, 2,398 had AA between 2009-2020, for an overall incidence of 13.6 cases per 100,000 patient-years. The incidence rate of AA by age was normally distributed and peaked at 6 years of age. Rates were 22.8% higher in female patients than in male patients. In addition, incidence rates were highest among Hispanics (31.5/100,000 person-years), followed by Asians (23.1/100,000 person-years), Blacks (17.0/100,000 person-years), and Whites (8.8/100,000).

Logistic regression analysis showed general agreement with the unadjusted incidence data. Males were less likely to be diagnosed with AA, compared with females (adjusted odds ratio, 0.80; P < .001). Analysis across race/ethnicity revealed significantly increased rates among children from minority backgrounds when compared with white children. Hispanic children had the greatest risk of developing AA (aOR, 3.07), followed by Asian children (aOR, 2.02), and Black children (aOR, 1.73) (P < .001 for all associations). Patients with atopic dermatitis, thyroid disease, psoriasis, vitiligo, and trisomy 21 prior to AA diagnosis all had a significantly higher risk of developing AA, compared with those without those diagnoses.

“This is the largest description of pediatric AA to date,” Ms. McKenzie said. “The prevalence has increased steadily, with a twofold increase over the last 10 years, which mirrors other autoimmune disorders. Children who identify as Hispanic, Asian, and Black have significantly higher incidence rates of alopecia areata compared to those who identify as White.”

Moving forward, she added, “efforts should focus on increasing education and awareness of AA in diverse communities and in community pediatricians so that patients can be diagnosed correctly early on. We can also use this data to ensure that representative populations are included in clinical trials for patients with AA.”

Asked to comment on the results Maria Hordinsky, MD, professor and chair of the department of dermatology at the University of Minnesota, Minneapolis, said that the study “is a great contribution to our understanding of the epidemiology of pediatric alopecia areata and also highlights how common alopecia areata is in children.” In an interview, she said that it would be interesting to see if this is a worldwide phenomenon or unique to the United States.

Lawrence J. Green, MD, clinical professor of dermatology at George Washington University, Washington, who was asked to comment on the study, characterized the work as being “very informative. Looking at a large cohort of pediatric patients with alopecia areata diagnosed by a dermatologist or two or more nondermatologists, the authors found a higher incidence and prevalence in nonwhite children here in the United States. I am worried in fact, the true incidence could be even higher than noted in the searched database because nonwhite children can often come from underserved and undercared for areas.”

The other authors were Christopher B. Forrest, MD, PhD, Mitchell Maltenfort, PhD, and Leslie Castelo-Soccio, MD, PhD, of Children’s Hospital of Philadelphia. Dr. Castelo-Soccio is a consultant for Pfizer; the other authors reported having no financial disclosures. Dr. Hordinsky disclosed receiving grant support for clinical research work on hair diseases from Pfizer, Eli Lilly, Concert Pharmaceuticals, and Target Derm and grant support from the National Alopecia Areata Foundation; and is on an advisory panel for Cassiopea. Dr. Green disclosed that he is a speaker, consultant, or investigator for numerous pharmaceutical companies.

*This story was updated on 7/19/21.

dbrunk@mdedge.com

The prevalence of pediatric alopecia areata (AA) in the United States has increased twofold over the past decade and it disproportionately affects females and Hispanic children, according to results from the largest study to date on the topic.

“Alopecia areata is a relatively common cause of nonscarring hair loss in children,” Paige McKenzie said during the annual meeting of the Society for Pediatric Dermatology. “The only two epidemiologic studies that have been performed in children have been based on registry or survey data which is inherently at risk for bias,” she added, referring to studies published in 2017 and 2018. “Additionally, epidemiologic descriptions of alopecia areata in adults are limited and overall estimates have varied from 0.2% to 2%. Current understanding is also largely based on population studies in Olmsted County, Minnesota, an area with mostly White racial demographics, so it’s not representative of the U.S. population as a whole.”

To identify the incidence and prevalence of pediatric AA over time, and across age, race/ethnicity, and sex, Ms. McKenzie and colleagues conducted a retrospective cohort study from 2009 to 2020 using PEDSnet, a network of seven U.S. pediatric health institutions with a database of more than 6.5 million children. “PEDSnet is unique because it uses a common data model to standardize EHR data across different health systems and uses SNOMED [Systematized Nomenclature of Medicine]–Clinical Terms to identify specific patient populations,” said Ms. McKenzie, who was a clinical research fellow in the section of dermatology at the Children’s Hospital of Philadelphia during the 2020-2021 academic year.

She and her coauthors limited their analysis to children younger than age 18 who were assigned a SNOMED code for AA during at least one dermatology physician visit or at least two nondermatology physician visits. They also identified an incidence cohort that was a subset of the study cohort who had at least 12 months of follow-up. “To determine the accuracy of AA patient identification, we also reviewed 100 cases at random from one institution with a threshold of greater than 95% accuracy,” said Ms. McKenzie, who is now a fourth-year medical student at the University of Texas Southwestern Medical Center, Dallas.

Of 5,409,919 children included in the study, 5,801 had AA, for an overall prevalence of 0.11%. The prevalence doubled from 0.04% in 2009 to 0.08% in 2019. “It fell in 2020, which we believe is a result of the COVID-19 pandemic’s effects on health care utilization,” she said. AA prevalence peaked at 9 years of age and was higher among females, compared with males (0.12% vs. 0.09%, respectively). The prevalence was highest among Hispanic children (0.23%), followed by Asian children (0.17%), Black children (0.12%), and White children (0.08%).

The incidence cohort consisted of 2,896,241 children. Of these, 2,398 had AA between 2009-2020, for an overall incidence of 13.6 cases per 100,000 patient-years. The incidence rate of AA by age was normally distributed and peaked at 6 years of age. Rates were 22.8% higher in female patients than in male patients. In addition, incidence rates were highest among Hispanics (31.5/100,000 person-years), followed by Asians (23.1/100,000 person-years), Blacks (17.0/100,000 person-years), and Whites (8.8/100,000).

Logistic regression analysis showed general agreement with the unadjusted incidence data. Males were less likely to be diagnosed with AA, compared with females (adjusted odds ratio, 0.80; P < .001). Analysis across race/ethnicity revealed significantly increased rates among children from minority backgrounds when compared with white children. Hispanic children had the greatest risk of developing AA (aOR, 3.07), followed by Asian children (aOR, 2.02), and Black children (aOR, 1.73) (P < .001 for all associations). Patients with atopic dermatitis, thyroid disease, psoriasis, vitiligo, and trisomy 21 prior to AA diagnosis all had a significantly higher risk of developing AA, compared with those without those diagnoses.

“This is the largest description of pediatric AA to date,” Ms. McKenzie said. “The prevalence has increased steadily, with a twofold increase over the last 10 years, which mirrors other autoimmune disorders. Children who identify as Hispanic, Asian, and Black have significantly higher incidence rates of alopecia areata compared to those who identify as White.”

Moving forward, she added, “efforts should focus on increasing education and awareness of AA in diverse communities and in community pediatricians so that patients can be diagnosed correctly early on. We can also use this data to ensure that representative populations are included in clinical trials for patients with AA.”

Asked to comment on the results Maria Hordinsky, MD, professor and chair of the department of dermatology at the University of Minnesota, Minneapolis, said that the study “is a great contribution to our understanding of the epidemiology of pediatric alopecia areata and also highlights how common alopecia areata is in children.” In an interview, she said that it would be interesting to see if this is a worldwide phenomenon or unique to the United States.

Lawrence J. Green, MD, clinical professor of dermatology at George Washington University, Washington, who was asked to comment on the study, characterized the work as being “very informative. Looking at a large cohort of pediatric patients with alopecia areata diagnosed by a dermatologist or two or more nondermatologists, the authors found a higher incidence and prevalence in nonwhite children here in the United States. I am worried in fact, the true incidence could be even higher than noted in the searched database because nonwhite children can often come from underserved and undercared for areas.”

The other authors were Christopher B. Forrest, MD, PhD, Mitchell Maltenfort, PhD, and Leslie Castelo-Soccio, MD, PhD, of Children’s Hospital of Philadelphia. Dr. Castelo-Soccio is a consultant for Pfizer; the other authors reported having no financial disclosures. Dr. Hordinsky disclosed receiving grant support for clinical research work on hair diseases from Pfizer, Eli Lilly, Concert Pharmaceuticals, and Target Derm and grant support from the National Alopecia Areata Foundation; and is on an advisory panel for Cassiopea. Dr. Green disclosed that he is a speaker, consultant, or investigator for numerous pharmaceutical companies.

*This story was updated on 7/19/21.

dbrunk@mdedge.com