Erythema elevatum diutinum

29 September 2015, by WALLACH D. & VIGNON-PENNAMEN M-D.

Erythema elevatum diutinum is a rare skin disease characterised by its chronicity. Although today it is still classified as cutaneous vasculitis, recent publications confirm its attachment to the group of neutrophilic dermatoses [6].

Erythema elevatum diutinum is characterized by dark red, purple or yellow plaques, papules or nodules. The lesions have a firm consistency with a decidedly annular configuration and are distributed symmetrically on the extensor surfaces of the limbs and preferably around the joints of the hands, elbows and knees. The buttocks and the Achilles tendon area are also frequently affected. This skin condition can be accompanied by sensations of itching or burning.

This dermatosis is most often isolated. It is sometimes accompanied by arthralgia, myalgia, more rarely by ocular lesions [5] and even, like in other neutrophilic dermatoses, by deep-seated aseptic neutrophilic lesions, particularly affecting the lungs.

The histological diagnosis is of varying difficulty depending on the stage of the disease [7]. With recent lesions, small-vessel vasculitis of the entire dermis is easily recognized. Later, fibrosis dominates, obliterating the vascular changes and the infiltrate which may lead to misdiagnosis. The xanthomatous lesions correspond to intra- rather than extracellular cholesterolosis. Direct immunofluorescence studies give varying, often negative results.

Among the diseases associated with erythema elevatum diutinum, monoclonal gammopathies, especially of the IgA type, are the most common. The association with inflammatory bowel disease, rheumatoid arthritis and systemic vasculitis (Wegener’s disease, relapsing polychondritis) is probably not accidental, similarly to the few observations of myelodysplastic syndromes occurring during the progression of erythema elevatum diutinum.

The association with HIV infection is noteworthy [4]. The clinical presentation is often specific with nodular exuberant lesions of the extremities that make it difficult to differentiate from Kaposi’s sarcoma and bacillary angiomatosis.

The etiopathogenesis of erythema elevatum diutinum is unknown. Certain facts, such as streptococcal hypersensitivity, the cases associated with HIV infection, a histological picture of vasculitis, point to the implication of an immunological reaction related to immune complexes. In some cases, a disorder of neutrophil function has been demonstrated. The detection of IgA antineutrophil cytoplasmic antibodies seems to be a marker for diagnosis. Although links exist between neutrophils and IgA disorders, their roles remain speculative.

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