Pollmann Robert, Eming Rüdiger
Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany.
Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany.
J Invest Dermatol. 2017 Jan;137(1):e1-e6. doi: 10.1016/j.jid.2016.11.003.
Autoimmune blistering diseases are examples of autoantibody-mediated, organ-specific autoimmune disorders. Based on a genetic susceptibility, such as a strong HLA-class II association, as yet unknown triggering factors induce the formation of circulating and tissue-bound autoantibodies that are mainly directed against adhesion structures of the skin and mucous membranes. Compared with other autoimmune diseases, especially systemic disorders, the pathogenicity of autoimmune blistering diseases is relatively well described. Several animal models of autoimmune blistering diseases have been established that helped to uncover the immunological and molecular mechanisms underlying the blistering phenotypes. Each in vivo model focuses on specific aspects of the autoimmune cascade, from loss of immunological tolerance on the level of T and B cells to the pathogenic effects of autoantibodies upon binding to their target autoantigen. We discuss current mouse models of autoimmune blistering diseases, including models of pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, and dermatitis herpetiformis.
自身免疫性大疱性疾病是自身抗体介导的器官特异性自身免疫性疾病的实例。基于遗传易感性，如与 HLA - II 类分子的强关联，目前尚不清楚的触发因素会诱导循环和组织结合的自身抗体形成，这些自身抗体主要针对皮肤和粘膜的黏附结构。与其他自身免疫性疾病，尤其是全身性疾病相比，自身免疫性大疱性疾病的致病性描述得相对清楚。已经建立了几种自身免疫性大疱性疾病的动物模型，这些模型有助于揭示水疱形成表型背后的免疫和分子机制。每个体内模型都关注自身免疫级联反应的特定方面，从 T 细胞和 B 细胞水平的免疫耐受丧失到自身抗体与其靶自身抗原结合后的致病作用。我们讨论了目前自身免疫性大疱性疾病的小鼠模型，包括寻常型天疱疮、大疱性类天疱疮、获得性大疱性表皮松解症和疱疹样皮炎的模型。
