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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.
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.
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.
FROM SISD 2021