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This rare condition affects both men and women. The average age at onset is 53 years. The lesions are deep brownish red to purple papules, nodules, and plaques. Blisters and ulcers also can occur.
This rare condition affects both men and women. The average age at onset is 53 years.1 The lesions are deep brownish red to purple papules, nodules, and plaques. Blisters and ulcers also can occur, as pictured here.
Eruptions may be seen anywhere on the body but are often found on the extensor surfaces of the extremities and, particularly, the joints. Erythema elevatum diutinum lesions can clinically resemble those of Kaposi's sarcoma and Sweet's syndrome.
Most likely, this disease is caused by immune complex deposition (Arthus reaction) in the dermal vessels. Excessive exposure to antigens, as from recurrent infections, or situations in which high levels of antibody occur (paraproteinemias) are likely to cause immune complexes with subsequent vessel deposition. Medical problems, such as myeloproliferative disorders and chronic or recurrent infections, also may be associated with the condition.
Histologically, erythema elevatum diutinum represents a localized vasculitis accompanied by a rich neutrophilic and histiocytic infiltrate. Dermal fibrosis and lipid deposits may be seen in older lesions.
Dapsone is the treatment of choice.
REFERENCE:1. Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 3rd ed. St Louis: Mosby; 1996:591.