天疱疮发病机制的研究进展

Pathogenesis of pemphigus

天疱疮是自身免疫引起表皮棘层细胞松解导致的慢性复发性表皮内大疱性皮肤病，典型表现为红斑基础上的疱壁松弛性水疱、糜烂、尼氏征阳性。依据天疱疮的临床表现，分为寻常型、增殖型、落叶型和红斑型天疱疮。天疱疮主要的发病机制是患者存在针对角质形成细胞桥粒芯（糖）蛋白的自身抗体，但棘层松解的详细机制尚不清。近年来，随着对蛋白组学、免疫学和分子生物学技术的发展以及天疱疮发病机制研究的不断深入，发现天疱疮的发病机制中除传统的桥粒芯（糖）蛋白自身抗体外，非桥粒芯（糖）蛋白抗体因素也参与了棘层松解的形成，为天疱疮提供了新的潜在治疗靶位。

Pemphigus is a chronic recurrent intraepidermal blistering skin disease caused by autoimmune response-induced epidermal acantholysis. It is characterized clinically by flaccid, thin-walled bullae arising on the erythematous skin, erosions and positive Nikolsky＇ s sign. According to clinical manifestations, pemphigus can be classified into pemphigus vulgaris （PV）, pemphigus vegetans （PVe）, pemphigus foliaceus （PF） and pemphigus erythematous （PE）. The main pathogenesis of pemphigus is the presence of autoantibodies against keratinocyte desmogleins （Dsgs） in patients, but exact mechanisms leading to acantholysis are still unclear. With the development of proteomies, immunology, molecular biological techniques and further insights into the pathogenesis of pemphigus in the past decades, it has been shown that besides anti-Dsg auto antibodies, non-Dsg antibodies are also involved in the formation of acantholysis, which provids new potential therapeutic targets for pemphigus.