Erythema Elevatum Diutinum

Abstract

Erythema elevatum diutinum (EED) is a chronic cutaneous vasculitis occurring in association with a variety of conditions including autoimmunity, infectious disease, and hematological abnormalities. The role of associated medical problems is controversial, and the exact pathogenesis of EED is unknown. A series of six cases is reported. The typical clinical presentation was that of erythematous papules and plaques involving the extensor surfaces of the extremities. Histologically, a spectrum from leukocytoclastic vasculitis to vessel occlusion and dermal fibrosis was seen. The lesions of EED have many mimics clinically and histologically. Establishing the diagnosis of EED is important so appropriate screening for associated conditions can ensue. The vascular endothelium of EED stains positive for CD31, CD34, VEGF, and factor VIIIa and negative for factor XIIIa, TGFB, and LANA, a reaction pattern that does not distinguish it from similar appearing lesions. Thus, the chronic and recurrent nature of EED is the primary means of distinguishing it from entities that are clinically and histologically similar.

A rare case of erythema elevatum diutinum presenting as diffuse neuropathy

“…Constitutional symptoms, including fever and arthralgias, are often seen. The disease course is chronic with frequent relapses 8 . Our patient's clinical presentation and history were consistent.…”

“…The lesions are generally asymptomatic but might be tender, pruritic, or painful. Lesions are initially soft and become more firm and indurated over time 8 . Constitutional symptoms, including fever and arthralgias, are often seen.…”

Erythema elevatum diutinum–associated with loss of the uvula

“…Patients are often systemically well, but symptoms such as arthralgia and myalgia may occur in some cases 2 . Histologically, a spectrum from LCV to vessel occlusion and dermal fibrosis may be observed 3 . EED may occur at any age, typically affecting adults age 30 to 60, although 1 case series reported 2 peaks of incidence: 1 in the sixth decade with an equal sex ratio and 1 in childhood with a greater incidence in females 4 .…”

“…EED occurs in association with a number of conditions including hematologic disease (IgA gammopathy, multiple myeloma), infectious disease (tuberculosis, HIV, streptococcal infection), immunologic disease (rheumatoid arthritis, inflammatory bowel disease), and malignancy (squamous cell carcinoma, breast cancer, B-cell lymphoma) 1, 3. Screening for associated disease, particularly hematologic disorders, is crucial to ensure that appropriate treatment is initiated early.…”

“…However, colchicine, mycophenolate mofetil, hydroxychloroquine, tetracycline or sulphonamide antibiotics, systemic and intralesional corticosteroids, and cyclophosphamide may also be beneficial 1, 2, 3, 4, 5, 6, 7…”