Onder M, Gürer M A
Gazi University School Medicine, Department of Dermatology, Ankara, Turkey.
J Eur Acad Dermatol Venereol. 2001 Mar;15(2):126-36. doi: 10.1046/j.1468-3083.2001.00251.x.
Behçet's disease (BD) is a chronic, inflammatory multisystemic condition of unknown aetiology. It is clinically characterized by recurrent orogenital ulcerations and skin eruptions; ocular manifestations; arthritis; vasculitis and in some cases neurological and large vessel involvement. Aetiology has not been defined, but genetic, environmental, viral, bacterial and immunological factors have been proposed as causative agents. Treatment includes colchicine, thalidomide, steroids and immunosuppressive agents and it is based on the severity of systemic manifestations, such as central nervous system involvement, arterial aneurysms and thrombosis of the major veins. Mortality is related to major system involvement. In this article the different clinical features, the multiple faces of BD and a list of currently suspected aetiological factors of the disease are discussed, and treatment modalities summarized.
白塞病(BD)是一种病因不明的慢性炎症性多系统疾病。其临床特征为复发性口腔和生殖器溃疡、皮肤疹;眼部表现;关节炎;血管炎,在某些情况下还包括神经和大血管受累。病因尚未明确,但遗传、环境、病毒、细菌和免疫因素被认为是致病因素。治疗包括秋水仙碱、沙利度胺、类固醇和免疫抑制剂,治疗方案根据全身表现的严重程度而定,如中枢神经系统受累、动脉动脉瘤和主要静脉血栓形成。死亡率与主要系统受累有关。本文讨论了白塞病的不同临床特征、其多面性以及目前怀疑的该疾病病因因素列表,并总结了治疗方式。