Erythema Elevatum Diutinum

Updated: Jun 03, 2016
  • Author: Firas G Hougeir, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Erythema elevatum diutinum (EED) is a rare type of leukocytoclastic vasculitis characterized by red, purple, brown, or yellow papules, plaques, or nodules. These lesions are usually distributed on the extensor surfaces of the body. Erythema elevatum diutinum was first described in 1888 by Hutchinson [1] and in 1889 by Bury. [2] However, the name erythema elevatum diutinum was first used by Radcliff-Crocker and Williams [3] who found similarities between the cases of Hutchinson and Bury and their own. No clinical examination difference is apparent between the Hutchinson type and the Bury type of erythema elevatum diutinum. The difference lies in the patient population and possibly the cause of onset.

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Pathophysiology

The pathophysiology of erythema elevatum diutinum is not well understood. According to Gibson and Su, [4] the lesions are thought to be caused by the deposition of immune complexes in small blood vessels. This induces an inflammatory cascade, which damages the vessels. This repetitive damage causes fibrosis and the appearance of cholesterol crystals and myelin figures in the vessels. Direct immunofluorescence shows deposits of complement as well as immunoglobulin G (IgG), immunoglobulin (IgM), immunoglobulin A (IgA), [5] and fibrin around the damaged vessels. The findings from Grabbe et al [6] support the idea that the initiation of erythema elevatum diutinum may occur via activation of cytokines such as interleukin 8, which causes selective recruitment of leukocytes to tissue sites. [7]

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Epidemiology

Frequency

Erythema elevatum diutinum is a rare disease. Although first described in 1888, the largest study of erythema elevatum diutinum was published in 1992 and included 13 patients. [8]

Race

No racial predilection is reported for erythema elevatum diutinum.

Sex

Erythema elevatum diutinum is found in both males and females. The Hutchinson type of erythema elevatum diutinum is predominant in men. The Bury type of erythema elevatum diutinum is found in women with a history of rheumatologic disease.

Age

Erythema elevatum diutinum can occur at any age. However, erythema elevatum diutinum is mostly an adult disease that occurs from the third to sixth decade of life. Men with erythema elevatum diutinum are usually older (Hutchinson type), and women are usually younger (Bury type).

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Prognosis

Erythema elevatum diutinum is a chronic disease that usually evolves over a 5- to 10-year period, at which point it may resolve. Erythema elevatum diutinum lesions tend to not leave scars, but areas of hyperpigmentation or hypopigmentation can be visible.

No mortality due to erythema elevatum diutinum has been reported. Erythema elevatum diutinum lesions can be completely asymptomatic, but they may be cosmetically disturbing. In other cases, the lesions can be associated with pain, itching, and/or a burning sensation. The most common systemic symptom is joint pain.

Dapsone and other therapies can be successful in limiting the progression of the disease.

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