Behçet disease in children: a nationwide retrospective survey in Japan.

The purpose of the present study was to evaluate the incidence of Behçet disease among Japanese children and to compare their clinical features with adult patients. A first questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan. Fifty-one cases of Behçet disease among children under 16 years of age were reported. From a second survey, 31 cases satisfied the criteria of the Behçet's Syndrome Research Committee of Japan. Of 31 patients (14 boys and 17 girls), three cases were the complete type, 24 cases were the incomplete type, and four cases were the possible type. As to the specific type of the disease, 10 cases were of the entero-Behçet type, two were a combination of the entero-vasculo-Behçet type, and four were the neuro-Behçet type. In the first 6 months after onset, oral ulcers were observed in 77% of cases, genital ulcers in 45%, uveitis in 10%, and skin lesions were observed in 39% of cases. During the course of the disease, the frequency of oral ulcers was 100%, genital ulcers, 58%; ocular complications, 29%; and skin lesions, 55%. Gastrointestinal signs and symptoms were more frequent in childhood Behçet disease than in adults, while ocular complications were less frequent. The diagnosis of Behçet disease in children is difficult because of the long interval before the onset of enough manifestations to satisfy the diagnostic criteria. Specific diagnostic criteria are necessary, based on a discussion of the many childhood cases of Behçet disease.