Disseminated granuloma annulare-like presentation of juvenile Wells' syndrome

A 5-year-old boy was referred because of urticarial flares with angioedema. Antihistamines and systemic steroids were not useful to prevent flares. At examination, at distance of a flare, chronic red-brownish plaques were found on the legs, trunk, and back, resembling granuloma annulare. The skin biopsy showed epithelioid and giant-cell granulomas, elastophagia, and “flames figures” surrounded with many eosinophils. A diagnosis of disseminated granuloma annulare-like variant of Wells' syndrome as described earlier by Caputo was made. A treatment with dapsone was initiated and enabled to stop flares. Wells' syndrome is a rare clinicopathologic entity with a chronic benign course and often misleading clinical presentation. Our case illustrates the clinical polymorphism and benefit of dapsone therapy.