Zouboulis C C, Kurz K, Bratzke B, Orfanos C E
Universitäts-Hautklinik und Poliklinik, Klinikum Steglitz der Freien Universität Berlin.
Hautarzt. 1991 Jul;42(7):451-4.
Vascular involvement in Behçet's disease was first mentioned by Adamantiades and occurs in addition to the classic symptom triad. In the present work an unusually fulminant course of Adamantiades-Behçet disease with a lethal outcome is reported in a 43-year-old male patient of German origin. The first symptoms were painful oedemas of the joints, followed 1 year later by bipolar aphthous lesions and by multiple progressive ulcers of the skin and the oral mucosa. Histologically, pustule formation with underlying vasculitis was diagnosed. In spite of systemic treatment with azathioprine and high-dosed prednisolone, the course was progressive with multiple necrotizing ulcers, and the patient died 18 months after the onset of the disease with signs of heart insufficiency and cachexia. The autopsy findings revealed multiple lesions of ulcerating systemic vasculitis of the skin and other visceral organs, including the lungs and the central nervous system, and also endocarditis verrucosa ulcerosa of the mitral valve.
白塞病中的血管受累最早由阿达曼蒂亚德斯提及，且是在经典症状三联征之外出现。在本研究中，报道了一名43岁德国裔男性患者患阿达曼蒂亚德斯-白塞病的异常暴发性病程，并导致死亡。最初症状为关节疼痛性水肿，1年后出现双相口疮性病变以及皮肤和口腔黏膜的多处进行性溃疡。组织学检查诊断为伴有潜在血管炎的脓疱形成。尽管使用硫唑嘌呤和大剂量泼尼松龙进行了全身治疗，但病程仍呈进展性，出现多处坏死性溃疡，患者在疾病发作18个月后死于心力衰竭和恶病质迹象。尸检结果显示皮肤和其他内脏器官（包括肺和中枢神经系统）存在溃疡性系统性血管炎的多处病变，以及二尖瓣疣状溃疡性心内膜炎。
