PDF(Pubmed)
Abstract：
Pemphigus is a life-threatening autoimmune blistering disease affecting skin and mucous membranes. Despite its etiopathogenesis remains largely unknown, several trigger and predisposing factors have been reported. Pemphigus is caused by autoantibodies that target desmoglein 1 and desmoglein 3, impacting desmosome function. However, circulating autoantibodies are often the consequence of a precipitating factor that occurs in predisposed individuals. This review aims to describe and discuss almost all trigger and predisposing factors reported as possible or probable cause of the disease. Among the reported trigger factors that may induce or exacerbate pemphigus, we have found of particular interest: drug intake (especially thiol- and phenol-containing compounds), vaccines, infections, as well as some reports about pregnancy, radiations, emotional stress, pesticides and physical trauma. Moreover, we discuss the possible role of food intake in pemphigus onset and particular attention is given to dietary factors containing thiol, phenol and tannin compounds. A trigger factor is \"the straw that breaks the camel\'s back,\" and often acts together with predisposing factors. Here we discuss how pemphigus onset may be influenced by genetic susceptibility and comorbidities like thyroid diseases, malignancies and other autoimmune disorders. To identify other hitherto unknown trigger and predisposing factors, well designed prospective studies are needed. In this context, future research should explore their connection with the aim to advance our understanding of pemphigus pathogenesis.
摘要：
天疱疮是一种危及生命的自身免疫性起泡疾病，影响皮肤和粘膜。尽管其病因仍然很大程度上未知，已经报道了一些触发因素和诱发因素。天疱疮是由靶向桥粒蛋白1和桥粒蛋白3的自身抗体引起的，影响桥粒功能。然而,循环自身抗体通常是在易感个体中发生的沉淀因子的结果。这篇综述旨在描述和讨论几乎所有被报道为可能或可能的疾病原因的触发和诱发因素。在报告的可能诱发或加剧天疱疮的触发因素中，我们发现特别感兴趣：药物摄入（尤其是含硫醇和苯酚的化合物），疫苗,感染,以及一些关于怀孕的报道，辐射,情绪压力,杀虫剂和身体创伤。此外，我们讨论了食物摄入在天疱疮发病中的可能作用，并特别注意含有硫醇的饮食因素，苯酚和单宁化合物。一个触发因素是“打破骆驼背部的稻草”，“并经常与诱发因素一起起作用。在这里，我们讨论天疱疮的发病可能受到遗传易感性和甲状腺疾病等合并症的影响，恶性肿瘤和其他自身免疫性疾病。为了确定迄今为止未知的其他触发因素和诱发因素，需要精心设计的前瞻性研究。在这种情况下，未来的研究应该探索它们之间的联系,以提高我们对天疱疮发病机制的认识.
