Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 550 First Avenue, New York, NY 10016, USA.
Med Hypotheses. 2010 Apr;74(4):727-8. doi: 10.1016/j.mehy.2009.10.038. Epub 2009 Nov 28.
Bullous pemphigoid is an autoimmune blistering disease of the skin caused by autoantibodies directed against basement membrane zone adhesion molecules. Autoantibodies cannot fully explain several important features of the disease such as the difficulty transferring with the pathogenic autoantibodies, or the presence of heavy lesional infiltration of eosinophils and neutrophils that is necessary for disease production. There is increasing evidence that Th17 cells and the cytokines they release such as interleukin-17 are important regulators of innate and adaptive immune responses in many Th1 and/or Th2 mediated autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, and allergic asthma. There is also evidence that Th17 cells have a role in pathogenesis of blistering skin diseases. Interleukin-17 is important in initiation and maintenance of many autoimmune reactions and it is involved in production of pro-inflammatory cytokines, matrix metalloproteinases, neutrophils, and eosinophils, all of which are important pathogenic factors in bullous pemphigoid. The hypothesis is that interleukin-17 has an important pathogenic role in BP and can describe features of the disease not explained by the autoantibody theory. This cytokine can be assessed in the blister fluid and sera of patients, and can be used as a marker of disease activity and response to therapy. The information obtained could also lead to the development of novel therapeutic strategies for this and other autoimmune blistering diseases.
大疱性类天疱疮是一种自身免疫性水疱病，由针对基底膜带黏附分子的自身抗体引起。自身抗体不能完全解释该疾病的几个重要特征，例如难以与致病性自身抗体转移，或病变部位浸润大量嗜酸性粒细胞和中性粒细胞，而这些细胞对于疾病的发生是必需的。越来越多的证据表明，Th17 细胞及其释放的细胞因子，如白细胞介素-17，是许多 Th1 和/或 Th2 介导的自身免疫性疾病（如类风湿关节炎、系统性红斑狼疮和过敏性哮喘）中固有和适应性免疫反应的重要调节剂。也有证据表明，Th17 细胞在水疱性皮肤病的发病机制中起作用。白细胞介素-17 在许多自身免疫反应的启动和维持中起着重要作用，它参与促炎细胞因子、基质金属蛋白酶、中性粒细胞和嗜酸性粒细胞的产生，所有这些都是大疱性类天疱疮的重要致病因素。假设白细胞介素-17 在 BP 中具有重要的致病作用，可以描述自身抗体理论无法解释的疾病特征。这种细胞因子可以在患者的水疱液和血清中进行评估，并可用作疾病活动和治疗反应的标志物。所获得的信息还可能为这种疾病和其他自身免疫性水疱病的治疗策略的发展提供依据。
