Behçet’s syndrome in pediatric age

A. Mendes, S. Braga, C. Vilarinho, M. A. Costa, C. Ferreira, T. Simão
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Abstract

Introduction: Behcet's syndrome is a systemic vasculitis characterized by recurrent oral and/or genital ulcers, and several systemic manifestations. The authors describe the case of a pediatric-onset Behcet's syndrome. Case report: An 11-year-old boy was referred to the Pediatric consultation after two episodes of great saphenous vein thrombophlebitis. He had experienced daily oral aphthae for the past three years, and various episodes of folliculitis with pustule formation. Laboratory study was normal. The boy showed no signs of uveitis. The diagnosis of Behcet's syndrome diagnosis was established according to the international criteria, with positive HLA- B51 testing. Colchicine was initiated, with favourable response. Conclusions: Due to clinical feature overlap with other conditions, Behcet's syndrome diagnosis remains challenging. Consensus pediatric classification criteria developed in 2016 enabled greater sensitivity and earlier diagnosis.
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儿童时期的behaperet综合征
白塞综合征是一种全身性血管炎,以复发性口腔和/或生殖器溃疡和几种全身性表现为特征。作者描述了一个儿科发病白塞综合征的病例。病例报告:一名11岁男孩在两次大隐静脉血栓性静脉炎发作后被转到儿科会诊。在过去的三年里,他每天都有口腔溃疡,并有多次毛囊炎和脓疱形成。实验室检查正常。这男孩没有表现出葡萄膜炎的迹象。根据国际标准建立白塞综合征诊断,HLA- B51检测阳性。开始使用秋水仙碱,反应良好。结论:由于与其他疾病的临床特征重叠,白塞综合征的诊断仍然具有挑战性。2016年制定的共识儿科分类标准提高了敏感性和早期诊断。
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