Uziel Y, Brik R, Padeh S, Barash J, Mukamel M, Harel L, Press J, Tauber T, Rakover Y, Wolach B
Department of Pediatrics, Meir General Hospital, Sapir Medical Center, Israel.
Clin Exp Rheumatol. 1998 Jul-Aug;16(4):502-5.
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 of BD among Israeli children.
A questionnaire was sent to 8 pediatric rheumatology units in Israel and 30 cases of BD diagnosed before the age of 16 years were identified.
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.
Juvenile BD in Israel is not uncommon, and is frequently associated with HLA B5 positivity. This could indicate a genetic susceptibility in our region. Half of the patients in our series had an incomplete form of BD, which may represent a less severe variant of the disease. In any case, careful follow-up is required, since their condition could eventually evolve into complete BD.
白塞病（BD）是一种主要见于年轻成年男性的血管炎。青少年BD较为罕见，仅有少量儿科病例系列报道。本研究的目的是明确以色列儿童中BD的流行病学及临床特征。
向以色列的8个儿科风湿病科单位发放问卷，共识别出16岁之前诊断为BD的30例病例。
15例患者符合国际研究组的BD诊断标准，另外15例为不完全型BD。在完全型BD患者中，口腔炎和皮肤受累是最常见的表现。其他症状包括生殖器溃疡、葡萄膜炎、中枢神经系统受累、关节炎及胃肠道受累。3例患者有阳性家族史。12例患者中有7例（58%）检测到HLA B5。15例不完全型BD患者均有复发性口腔炎；其他表现包括葡萄膜炎、关节炎及生殖器溃疡。该组患者中94%检测到HLA B5。
以色列青少年BD并不少见，且常与HLA B5阳性相关。这可能表明我们地区存在遗传易感性。本系列研究中半数患者为不完全型BD，这可能代表疾病的一种较轻变异型。无论如何，均需密切随访，因为其病情最终可能进展为完全型BD。
