Erythema elevatum diutinum

Erythema elevatum diutinumDescription, Causes and Risk Factors:ICD-10: L95.1.Abbreviation: EED.Erythema elevatum diutinum (EED) is a chronic and rare dermatosis that is considered to be a variant of leukocytoclastic vasculitis. It is probably mediated by immune complexes. It is generally associated with autoimmune, neoplastic, and infectious processes. The disease may occur in any age group, but patients are typically in their third, fourth, or fifth decade. The incidence is equal in men and women.Both hematologic and non-hematologic abnormalities have been associated with EED. Hematologic abnormalities include myelodysplasia, multiple myeloma, IgA paraproteinemia and polycythemia vera. Non-hematologic abnormalities include systemic lupus erythematosus and HIV.The pathogenesis is still unknown; it is supposed to be an immune complex vasculitis. EED may be associated with autoimmune diseases, infections and malignancies like B cell lymphoma, breast carcinoma and can precede hypergammaglobulinemia, IgA monoclonal gammopathy, myelodysplasia and multiple myeloma by many years. It is also associated with longstanding verrucous hyperplasia, chronic ulcers, draining sinuses of hidradenitis suppurativa, genital lichen sclerosus; and uncommonly, with hypertrophic lichen planus.Symptoms:Erythema elevatum diutinumThe typical clinical presentation is that oferythematous papules and plaques involving theextensor surfaces of the extremitiesand areusually located near joints such as the fingers,hands, elbows, ankles, and knees. However, on rare occasions, blisters and ulcers may also appear.Although skin is the major organ involved, systemic symptoms such as arthralgias can be present and quite severe.These symptoms can be exacerbated by cold.Diagnosis:The diagnosis is based on the characteristic morphology, distribution pattern of the lesions and histopathological findings of leukocytoclastic vasculitis. A thorough investigative work-up of the patients is important to detect the systemic association especially myeloproliferative disorders which can be life-threatening.Skin biopsy is usually diagnostic; other investigations like direct immunofluorescence and electron microscopy can be done in case of doubt.Treatment:Treatment of EED is difficult because this disorder runs a chronic and recurrent course. Treatment of the underlying cause or infection should result in improvement of EED. In most cases, dapsone or sulfonamides are considered the first-line treatment for EED. Other drugs that have been used include niacinamide, colchicine, chloroquine, clofazimine and cyclophosphamide. Oral corticosteroids are generally ineffective. Surgical excision of the lesions is sometimes performed to provide symptomatic relief.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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