Burrows N P, Zhao M H, Norris P G, Lockwood C M
Department of Dermatology, Addenbrooke's Hospital Cambridge, England.
J R Soc Med. 1996 Jan;89(1):47P-8P. doi: 10.1177/014107689608900113.
Behçet's disease is a multisystem disorder affecting primarily mucocutaneous and ocular sites although the gastrointestinal, cardiovascular, central nervous and respiratory systems may also be involved. Hulusi Behçet, a Turkish dermatologist, first described Behçet's disease in 1937 and suggested a possible infectious aetiology. The pathogenesis of this condition still remains unclear although it is likely that infection acts as a trigger in genetically susceptible individuals. We report a patient with unusual cutaneous manifestations of Behçet's disease and antineutrophil cytoplasmic antibodies (ANCA) directed against the cytotoxic protein, bactericidal/permeability-increasing protein (BPI). This is the first report of Behçet's disease associated with this autoantibody.
白塞病是一种多系统疾病，主要累及黏膜皮肤和眼部，尽管胃肠道、心血管、中枢神经和呼吸系统也可能受累。土耳其皮肤科医生胡卢西·白塞于1937年首次描述了白塞病，并提出了可能的感染病因。尽管感染可能在遗传易感个体中起触发作用，但这种疾病的发病机制仍不清楚。我们报告了一名患有不寻常皮肤表现的白塞病患者，其抗中性粒细胞胞浆抗体（ANCA）针对细胞毒性蛋白、杀菌/通透性增加蛋白（BPI）。这是白塞病与这种自身抗体相关的首例报告。
