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AT-A-GLANCE

AT-A-GLANCE

  • Rare disorder, with around 250 case reports in literature.

  • A chronic leukocytoclastic vasculitis typified by a distinctive clinical pattern of symmetric, erythematous, violaceous, or yellow–brown papules, nodules, or plaques.

  • Sites of involvement often include the skin overlying the joints of the hands and fingers, or extensor surfaces like the elbows, knees, legs, and Achilles tendon. The trunk is usually spared.

  • Coexisting diseases include monoclonal paraproteinemia, lymphoproliferative disorders, chronic infections, autoimmune conditions, and connective tissue disease.

  • Histopathology reveals a leukocytoclastic vasculitis in early-stage disease, and mixed inflammation and fibrosis in late-stage disease.

Erythema elevatum diutinum (EED) is a rare, chronic leukocytoclastic vasculitis (LCV) that presents as largely symmetric papules and plaques, chiefly over joints and upon extensor surfaces, with a pink, erythematous, brown, violaceous, or yellow hue. Cases now recognized as EED were first reported by Hutchinson1,2 in 1878, and Bury3 in 1889. The current nosology, EED, was proposed by Radcliffe-Crocker and Williams in 1894,4 with the last part of the name, diutinum, meaning “long-lasting.”

Radcliffe-Crocker and Williams proposed a division of cases into “Bury-type” (more often seen in young women with a personal and/or family history rheumatism), and “Hutchinson-type” (more often seen in older men with gout). In 1932, so-called extracellular cholesterosis of Urbach5 was proposed as a third form. In 1929, Weidman and Besancon classified EED as a vasculitis,6 and this conclusion is now widely held. EED is no longer subdivided, and all forms are considered a spectrum of the disorder.

EPIDEMIOLOGY

The incidence of EED is unknown, with only about 250 cases reported in the literature.7 A case series, culled from a tertiary care center, and spanning 60 years, contained only 13 cases.8 EED may present at any age but is most common in the fourth through sixth decades of life. One of the original cases reported occurred in a 6-year-old.2 Men were thought to be affected more often, but with so few cases reported, it is difficult to ascertain any definitive sexual or ethnic predilection.

CLINICAL FEATURES

EED typically first presents as edematous, erythematous to violaceous papules, nodules, and plaques, distributed relatively symmetrically upon the skin overlying the joints of the fingers, toes, and hands (Fig. 140-1), or upon the extensor surfaces, such as the elbows (Fig. 140-2), wrists, knees (Fig. 140-3), ankles, legs, and Achilles tendon. Purpura, petechiae, vegetative lesions,9 ulcerations, and bullous appearance may occur in association with EED.10

Figure 140-1

Erythema elevatum diutinum. Nodular lesions on dorsal hand present for several years.

Figure 140-2

Multiple erythematous scaly papules coalescing into plaques on extensor elbow.

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