[Behçet's disease in a Dutch boy with painful skin lesions]

Abstract

A 14-years-old Dutch boy had pain in both calves, recurrent oral ulcers, fever, hoarseness and erythema nodosum-like skin lesions. Laryngoscopic examination revealed a vocal cord ulcer and leukocytoclastic vasculitis was visible in a skin biopsy. Based on the clinical picture, the diagnosis of 'incomplete juvenile Behçet's disease' was established. During treatment with colchicine, genital ulcers developed. These disappeared after local treatment with corticosteroids. Juvenile Behçet's disease is rare in Western Europe and the diagnosis is often difficult. Diagnostic criteria have been formulated, but in most cases there is a delay before these criteria are fulfilled due to the slow clinical course of the disease. All organ systems may be affected; mucocutaneous and skin lesions are the most frequent manifestations. Therapy depends on the severity and the symptoms of the disease.