Behçet's syndrome (BS) presents in childhood in up to 20% of reported cases. Diagnosis is clinical and multiple classification criteria have been developed. Presentation is heterogenous with recurrent oral ulceration often being the presenting feature. Mucocutaneous disease including genital ulceration and skin involvement is a common phenotype. Vascular and neurological manifestations are rarer, particularly in childhood. Musculoskeletal and gastro-intestinal involvement which do not form part of commonly used classification criteria, appear more frequent in children. Treatment approaches are extrapolated from studies of adult onset disease. The pathogenesis of BS is not well defined although dysregulation in both innate and adaptive immune systems, together with abnormal antigen presentation have been described. The recent discovery of monogenic mimics of BS requires further genetic studies to understand the burden of monogenic autoinflammatory conditions affecting those with a BS phenotype.

中文翻译：

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青少年期白塞氏综合症和拟态。

贝塞特综合症（BS）的儿童期多达20％。诊断是临床，已经制定了多种分类标准。表现是异质的，复发性口腔溃疡通常是表现特征。包括生殖器溃疡和皮肤受累在内的皮肤粘膜疾病是常见的表型。血管和神经系统表现较为罕见，尤其是在儿童时期。肌肉骨骼和胃肠道受累并不构成常用分类标准的一部分，在儿童中似乎更为常见。治疗方法是从对成人发病的研究中推断出来的。尽管已经描述了先天性和适应性免疫系统的失调以及异常抗原呈递，但BS的发病机制尚不明确。