Clinical Reference / Therapeutic Strategies / Erythema Elevatum Diutinum

Erythema Elevatum Diutinum


Key Points

  • Erythema elevatum diutinum (EED) is a rare chronic form of fibrosing vasculitis.
  • Firm, red to brown or violaceous nodular lesions developing over joints of the elbows, knees, hands, and feet, often in a symmetric pattern, is the hallmark cutaneous lesion.
  • EED has been associated with HIV/AIDS, hematologic disorders (IgA monoclonal gammopathy, multiple myeloma), celiac disease, Wegener’s granulomatosis, connective tissue disorders (especially systemic lupus erythematosus), and recurrent streptococcal infections.
  • Dapsone is the first-line therapy for EED.

Introduction

Erythema elevatum diutinum (EED) is rare and is thought of as a chronic, fibrosing vasculitis with firm, red to brown or violaceous, nodular lesions developing over joints of the elbows, knees, hands, and feet, often in a symmetric pattern. Though the etiology of the disease is not known, inflammation resulting from immune complex deposition is suspected. The diagnosis is made on the basis of clinical appearance and histopathologic findings. EED has been associated with HIV/AIDS, hematologic disorders (IgA monoclonal gammopathy, multiple myeloma), celiac disease, Wegener’s granulomatosis, connective tissue disorders (especially systemic lupus erythematosus), and recurrent streptococcal infections. In HIV/AIDS, nodules on the soles are characteristic. EED has been rarely associated with ocular complications, including peripheral ulcerative keratitis. It is a chronic condition with intermittent manifestations that may recur over the span of 10-25 years. The goal of treatment is to limit the development of new lesions, i.e., progression of disease. Once lesions are advanced with evidence of fibrosis, they may not be amenable to treatment with anti-inflammatory agents.

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