Gallizzi Romina, De Vivo Dominique, Valenti Simona, Pidone Caterina, Romeo Carmelo, Caruso Rosario, Romano Claudio
Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatrics, University of Messina, Messina, Italy.
Department of Human Pathology in Adulthood and Childhood "G. Barresi", Unit of Pediatric Surgery, University of Messina, Messina, Italy.
Ital J Pediatr. 2017 Apr 7;43(1):33. doi: 10.1186/s13052-017-0350-3.
Behcet's disease (BD) is a chronic immune-mediated, inflammatory disorder which may affect a number of different systems (oral and genital mucosa, eyes, skin, vascular district, joints, gastrointestinal tract and nervous system). Neurological manifestations are present in 5-10%, and gastrointestinal tract involvement in 10-15% of cases. The simultaneous involvement of two systems, neurological and gastrointestinal tract, is very rare and represents the aim of our case report.
We describe a case of a 12-year-old girl with neurological (endocranial hypertension, papilledema, retinal vasculitis) and gastrointestinal tract (terminal ileum and cecum inflammation) involvement and with a history of recurrent oral aphthosis; therefore, according to both International Criteria for Behcet's Disease (ICBD) and Paediatric Behcet's Disease criteria (PEDBD) the diagnosis of BD was confirmed.
This case report is one of the few described in literature with simultaneous involvement of the two systems, neurological and gastrointestinal tract, in paediatric BD. The diagnosis is really difficult because there is no specific diagnostic test. We think that our clinical case should help clinicians to suspect a BD with an unusual onset.
白塞病（BD）是一种慢性免疫介导的炎症性疾病，可累及多个不同系统（口腔和生殖器黏膜、眼睛、皮肤、血管区域、关节、胃肠道和神经系统）。神经系统表现见于5% - 10%的病例，胃肠道受累见于10% - 15%的病例。神经系统和胃肠道两个系统同时受累非常罕见，这也是我们本病例报告的目的所在。
我们描述了一名12岁女孩的病例，她同时出现神经系统（颅内高压、视乳头水肿、视网膜血管炎）和胃肠道（回肠末端和盲肠炎症）受累，并有复发性口腔溃疡病史；因此，根据国际白塞病诊断标准（ICBD）和儿童白塞病标准（PEDBD），确诊为白塞病。
本病例报告是文献中少数描述儿童白塞病同时累及神经系统和胃肠道两个系统的病例之一。由于没有特异性诊断检查，诊断确实困难。我们认为我们的临床病例应有助于临床医生怀疑不寻常起病的白塞病。
