WELLS SYNDROME

By Gary M. White, MD

Wells Syndrome


Eosinophilic cellulitis or Wells syndrome (WS) is characterized by inflammatory papules and plaques that on biopsy show an abundance of eosinophils and so called flame figures. The flame figures result from extensive deposition of eosinophil granule major basic protein on collagen.

Clinical

Acute attacks of erythematous plaques--resembling cellulitis-- are characteristic. Lesions may occur anywhere, e.g., trunk, extremities, face. Initial erythema may be replaced by a blue-gray color. There may be significant underlying edema. Rarely, blisters may occur. Multiple recurrences over many years is common. Associated findings in primary, familial Well’s syndrome include blood eosinophilia, bone marrow eosinophilia, eosinophilic pleural effusions, and pericarditis. One family had lesions of eosinophilic cellulitis associated with mental retardation and an abnormal body habitus.

One of the most common triggers of WS is an insect bite. Erythematous papules, nodules and plaques may last months [PD 2017;33;677].

Workup

A CBC with eosinophil count, metabolic panel and skin biopsy should be done. If parasites are a possibility, then a stool exam and specific antibody titers are in order.

Treatment

Untreated lesions usually fade over 2-6 weeks. A potent topical steroid should be given. Systemic steroids are usually the most effective oral agent and may be given at doses of 1-2 mg/kg/day initially and tapered over several weeks. In one case of WS associated with colon cancer, hemicolectomy cured both the cancer and the WS. Steroid-sparing medications that have been reported effective include hydroxychloroquine, dapsone, minocycline, nicotinamide, azathioprine and cyclosporine.

Additional Pictures

Wells Syndrome

References

JAMA DERM

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