Clinical features of Behçet's disease in children

BACKGROUND: Behçet's disease (BD) is a systemic inflammatory vasculitis of young adults with unknown etiology. OBJECTIVE: The aim of this study was to determine the clinical features and outcome of BD in childhood. PATIENTS AND METHODS: Seventeen patients with BD within a pediatric age group were studied since 1997. RESULTS: Boys : girls ratio was 12:5. Familial cases were very frequent (45%). Oral aphthous ulcers were present in all patients (100%), genital aphthous ulcers were present in 16 (94%), ocular lesions in 4 (24%), erythema nodosum in 3 (18%), papulopustules in 8 (47%), joint symptoms in 13 (76%), mild gastrointestinal symptoms in 2 (12%), neurological involvement in 2 (12%), positive pathergy reactions in 13 (76%), and thrombophlebitis in 1 (6%). We used colchicine, systemic corticosteroids, cyclosporine, nonsteroidal anti-inflammatory drugs, sulfasalazine, and topical agents for the treatment of children with BD. CONCLUSION: The findings of BD in children are similar as in adults, but the frequency of familial cases is significantly higher.