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Erythema elevatum diutinum

Felipe Bochnia Cerci, MD
Joseph Jorizzo, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


Erythema elevatum diutinum (EED) is a chronic form of leukocytoclastic vasculitis consisting of violaceous, red-brown, or yellowish papules, plaques, or nodules that favor the extensor surfaces (picture 1A-D) [1,2]. EED may occur in association with infections, hematologic abnormalities, autoimmune diseases, or other conditions. Oral dapsone is the primary mode of treatment.

The clinical features, diagnosis, and management of EED will be reviewed here. Other manifestations of cutaneous leukocytoclastic vasculitis are reviewed separately. (See "Evaluation of adults with cutaneous lesions of vasculitis".)


EED is a rare disease that most frequently affects young and middle-aged adults between the ages of 30 and 60 years. There is no known sex or racial predilection [3].


The pathogenesis of EED is not well understood. EED appears to be a form of immune complex-mediated vasculitis. The cutaneous findings may result from the deposition of immune complexes in small blood vessels in the skin, leading to complement activation, neutrophilic infiltration, and the release of destructive enzymes [4]. In support of this theory, direct immunofluorescence studies reveal perivascular deposition of complement, IgG, IgM, IgA, and fibrin in EED [5].

In addition, the findings of an in vitro study suggest that activation of cytokines such as interleukin-8 contributes to selective recruitment of leukocytes to affected skin [6]. Antineutrophil cytoplasmic antibodies (ANCA) may also play a role in the pathogenesis of EED [7].

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Literature review current through: Dec 2017. | This topic last updated: Jan 12, 2017.
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