ERYTHEMA ELEVATUM DIUTINUM

By Gary M. White, MD


Erythema elevatum diutinum (EED) is a rare variant of small vessel vasculitis that presents with symmetric red-purple papulonodules on the extensor surfaces, especially the hands. Although chronic, there is no systemic vasculopathy, although arthralgias may be present. A monoclonal gammopathy (particularly IgA but also IgG), myeloma (particularly IgA myeloma) and hypergammaglobulinemia have also been associated. Less common associations include HIV, hematologic malignancies, infections etc.

Clinical

Clinically one sees purplish, red papules, nodules and/or plaque symmetrically on the dorsa of the hands, knuckles, the extensor forearms and elbow. Initially, the lesions are soft because of dermal edema and tissue destruction. Over time, they become fibrotic and hard to palpation. Larger nodules and rarely bullous lesions may occur. The buttocks and achilles tendon should also be examined for lesions. The patient may complain of a burning sensation from the lesions. Small, yellowish papules reminiscent of xanthomas have been reported.

Workup

A paraprotein should be excluded. A general workup for malignancy should be performed. The presence of antineutrophil cytoplasmic antibodies (ANCAs) has been reported to be a useful clinical marker for EED and should be measured.

Treatment

Dapsone is the treatment of choice. Other sulfonamides may also be used. Potent topical steroids may be tried. Systemic steroids are generally ineffective. Niacinamide and tetracycline have been used. Other agents reported effective include colchicine, chloroquine, phenformin, clofazimine, cyclophosphamide and intermittent plasma exchange [AD 1996;132;1360]. Some cases of EED associated with malignancy have cleared after the cancer is treated.

References

Multiple plaques on the hands. IJDVL 2013;79;238

Nodules on the feet and ankles. Indian J Dermatol 2014;59:592-4

Erythema elevatum diutinum in association with dermatitis herpetiformis. Indian Dermatol Online J. 2014 Jan;5(1):48-50.

RegionalDerm

Homepage | FAQs | Use of Images | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.