Behcet's disease is a chronic, relapsing, multisystem disease characte
rized by the clinical triad of genital ulcers, oral ulcers and ocular
involvement. Twelve Saudi children are presented, all of whom satisfie
d the international criteria for the classification of Behcet's diseas
e and whose initial manifestations appeared at or before the age of 16
years. The male-to-female ratio was 1.4:1. The mean age at onset was
11.5 years (range 7-16 years) and the mean duration of disease was 6.5
years (range 3-13 years). Oral ulcers were present in all patients (1
00%), genital ulcers in 11 patients (91%), ocular involvement in the f
orm of anterior and/or posterior uneitis in 6 patients (50%), skin man
ifestations in 10 patients (83%), musculoskeletal symptoms in 9 patien
ts (75%), and central nervous system involvement in 6 patients (50%).
One patient had thrombophlebitis and another had pulmonary artery aneu
rysm. No renal, cardiovascular or gastrointestinal abnormalities were
detected. The pathergy test was positive in 3/7 patients. HLA B5 (W51)
typing was positive in 5/10 patients. This report of juvenile Behcet'
s disease in Saudi children suggests that this multisystem disease has
an aggressive nature and should be considered in the differential dia
gnosis of childhood vasculitis in endemic areas.