Department of Dermatology, INSERM U 905, Rouen University Hospital, University of Q1 Rouen, Rouen, France.
Clin Dermatol. 2011 Jul-Aug;29(4):432-6. doi: 10.1016/j.clindermatol.2011.01.013.
Pemphigus is a rare autoimmune bullous disorder involving the skin and mucosa. The disease has a chronic course. It is characterized histologically by an intraepidermal cleavage and the production of pathogenic antibodies directed against different proteins of the desmosomes, which belong to the cadherin family. The diagnosis of the type of pemphigus is made on clinical features, the level of histologic cleavage, and the identification of the antigens recognized by circulating autoantibodies using immunoblot or ELISA analysis of serum. The epidemiology and clinical, histologic, and immunologic findings of pemphigus vulgaris, pemphigus foliaceus, pemphigus vegetans, and pemphigus herpetiformis are described.
天疱疮是一种罕见的自身免疫性大疱性疾病，涉及皮肤和黏膜。该病呈慢性病程。组织学上的特征是表皮内分裂和产生针对桥粒不同蛋白的致病性抗体，桥粒属于钙黏蛋白家族。天疱疮的类型诊断基于临床特征、组织学分裂程度以及通过免疫印迹或 ELISA 分析血清中识别的抗原来确定循环自身抗体。本文描述了寻常型天疱疮、落叶型天疱疮、增殖型天疱疮和疱疹样天疱疮的流行病学、临床、组织学和免疫学特征。
