Objective Behçet's disease (BD) is a vasculitis mainly observed in young adult males.
Juvenile BD is rare and only small series of pediatric cases have been reported.
The objective of this study was to define the epidemiology and clinical features ofBD among Israeli children.
Methods A questionnaire was sent to 8 pediatric rheumatology units in Israel and 30 cases ofBD diagnosed before the age of 16 years were identified.
Results Fifteen patients fulfilled the International Study Group Criteria for BD, while 15 had an incomplete form of BD.
Among the patients with complete BD, stomatitis and skin involvement were the most common manifestations.
Other symptoms included genital ulcers, uveitis, CNS involvement, arthritis, and gastrointestinal involvement.
A positive family history was elicited in 3 patients.
HLA B5 was found in 7 of 12 patients (58%). The 15 patients with incomplete BD all had recurrent stomatitis ; other manifestations included uveitis, arthritis, and genital ulcers.
HLA B5 was found in 94% of this group.
Mots-clés Pascal : Oculomucocutané Behçet syndrome, Enfant, Homme, Symptomatologie, Epidémiologie, Israël, Asie, Questionnaire, Déterminisme génétique, Système HLA, Peau pathologie, Stomatologie, Oeil pathologie, Appareil génital pathologie, Maladie système, Appareil circulatoire pathologie, Vaisseau sanguin pathologie
Mots-clés Pascal anglais : Behçet syndrome, Child, Human, Symptomatology, Epidemiology, Israel, Asia, Questionnaire, Genetic determinism, HLA-System, Skin disease, Stomatology, Eye disease, Genital diseases, Systemic disease, Cardiovascular disease, Vascular disease
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 98-0403108
Code Inist : 002B07. Création : 25/01/1999.