Gorini Francesca, Coi Alessio, Santoro Michele, Tonacci Alessandro, Sansone Francesco, Mariotti Elena Biancamaria, Donati Marta, Verdelli Alice, Nasca Maria Rita, Amerio Paolo, Antiga Emiliano, Barletta Emanuela, Caproni Marzia
Unit of Epidemiology of Rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council, 56124 Pisa, Italy.
Information Systems Unit, Institute of Clinical Physiology, National Research Council, 56124 Pisa, Italy.
Int J Mol Sci. 2025 Jun 24;26(13):6076. doi: 10.3390/ijms26136076.
Bullous pemphigoid (BP) and pemphigus vulgaris (PV) represent the most prevalent conditions among autoimmune bullous skin diseases, considered a major cause of severe morbidity and, in certain cases, mortality. The hallmark of the two diseases is the presence of autoantibodies directed against proteins located in the basement membrane of the skin, which determines the formation of blisters. In recent years, interest in the role of microbiota in relation to health-disease status has progressively increased. In particular, based on the gut-skin axis, accumulating evidence has emerged on the potential association between the composition and diversity of microbial communities in the gut, skin, and even in the oral cavity and the risk of developing BP and PV. Dysbiosis, characterized by a generally higher relative abundance of Firmicutes and a depletion of probiotics/beneficial species, might contribute to the pathogenesis of both diseases. Despite the still limited number of studies and the need for further large-scale multicenter studies, the knowledge gathered so far is suggestive of a novel modifiable risk factor representing a potential target for adjuvant treatments of these disabling and life-threatening conditions.
大疱性类天疱疮（BP）和寻常型天疱疮（PV）是自身免疫性大疱性皮肤病中最常见的病症，被认为是严重发病甚至在某些情况下导致死亡的主要原因。这两种疾病的标志是存在针对位于皮肤基底膜的蛋白质的自身抗体，这决定了水疱的形成。近年来，人们对微生物群在健康与疾病状态方面的作用的兴趣日益增加。特别是，基于肠-皮肤轴，越来越多的证据表明，肠道、皮肤甚至口腔中微生物群落的组成和多样性与发生BP和PV的风险之间存在潜在关联。以厚壁菌门相对丰度普遍较高和益生菌/有益菌种减少为特征的生态失调可能促成这两种疾病的发病机制。尽管目前的研究数量仍然有限，且需要进一步开展大规模多中心研究，但迄今为止积累的知识表明，存在一种新的可改变的风险因素，这代表了对这些致残性和危及生命的病症进行辅助治疗的潜在靶点。
