Pediatric-onset Behçet disease in Bahrain: report of nine cases and literature review.

BACKGROUND This report studies the clinical features of Behçet disease (BD) in children and compares our results with other international studies. METHODS We retrospectively reviewed patient data that included the clinical picture, HLA typing, and treatment in BD cases. RESULTS This study reviewed data from a total of nine children with BD. Median age at presentation was seven years, with a male to female ratio of 2:1. There was one patient who had Down's syndrome. Oral ulcers were present in all children, while genital ulcers were present in only 66% of cases. Skin manifestation was seen in 88% and uveitis in 55%. There was evidence of gastrointestinal (55%), neurological (55%), and musculoskeletal manifestations (77%). HLA B5 was positive in 66% of cases and 55% had positive family histories. Apart from gastrointestinal symptoms, our results were comparable with other studies. CONCLUSION Awareness of BD symptoms in the pediatric age group is crucial for early diagnosis and treatment. The coexistence of BD and Down's syndrome needs further genetic study, which may link these two major disorders.