[Behçet disease in children in France].

Abstract

Background: Behçet's disease is rare in children. The possibility of incomplete forms and the fact that the criteria for its diagnosis are not still agreed upon may explain why it could occur more frequently.

Population and methods: A questionnaire was sent to all 362 French pediatric units. It requested data on possible sufferers from Behçet's disease, including EEG, CSF examination, brain CT scan, coloscopy, HLA groups, age at the first symptom and age at diagnosis. Among the 24 questionnaires concerning a possible case of Behçet's disease, 15 patients satisfied the international criteria for a diagnosis of Behçet's disease.

Results: These 15 patients (10 boys and 5 girls) were Caucasians (n = 9) or Africans (n = 6). The first symptoms were seen at 2 months to 14 years (mean: 10 yrs). The diagnosis was made between 7 and 18 years of age (mean: 11 yrs). HLA antigen B5 was identified in 7 patients and HLA-B12 in 1 patient. The first symptoms were oral ulcers (10 patients) plus skin lesions, genital ulcers and headache. All 15 patients suffered from oral ulcers, 8 from genital ulcers, 12 from skin lesions, 11 from arthritis, 9 from neurologic abnormalities, 6 from abdominal pain and 7 from eye inflammation; 10 patients had a complete form with ocular, neurologic and/or vascular abnormalities while 5 patients had an incomplete form with cutaneo-mucosal and/or GI and/or articular involvement. Two patients had family histories of Behçet's disease.

Conclusions: Behçet's disease is not so exceptional in France. A better knowledge of its symptoms should result in its more frequent diagnosis in childhood.