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Previous studies using patients' samples and animal disease models elucidated the complement\u2010dependent and complement\u2010independent pathways of blister formation, the pathogenic roles of immune cells (T and B cells, macrophages, mast cells, neutrophils, eosinophils), and the pathogenicity of IgE autoantibodies in BP. This review introduces the recent progress on the mechanism behind the epitope\u2010spreading phenomenon in BP, which is considered to be important to understand the chronic and intractable disease course of BP, and the pathogenicity of anti\u2010BP230 autoantibodies, mainly focusing on studies that used active disease models. To clarify the pathogenesis of BP, the mechanism behind the breakdown of immune tolerance to BP antigens should be investigated. Recent studies using various experimental models have revealed important roles for regulatory T cells in the maintenance of self\u2010tolerance to COL17 and BP230 as well as in the suppression of inflammation triggered by the binding of antibodies to COL17. Notably, physical stresses such as trauma, thermal burns, bone fractures, irradiation and ultraviolet exposure, some pathologic conditions such as neurological diseases and hematological malignancies, and the use of dipeptidyl peptidase\u2010IV inhibitors and immune checkpoint inhibitors have been reported as triggering factors for BP. These factors and certain underlying conditions such as genetic background, regulatory T\u2010cell dysfunction or aging might synergistically affect some individuals and eventually induce BP. Further studies on the breakdown of self\u2010tolerance and on the identification of key molecules that are relevant to blister formation and inflammation may expand our understanding of BP's etiology and may lead to the development of novel therapeutic approaches.<\/jats:p>","DOI":"10.1111\/1346-8138.16654","type":"journal-article","created":{"date-parts":[[2022,11,22]],"date-time":"2022-11-22T11:21:18Z","timestamp":1669116078000},"page":"140-149","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":42,"title":["What's new in the pathogeneses and triggering factors of bullous pemphigoid"],"prefix":"10.1111","volume":"50","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3489-5418","authenticated-orcid":false,"given":"Hideyuki","family":"Ujiie","sequence":"first","affiliation":[{"name":"Department of Dermatology, Faculty of Medicine and Graduate School of Medicine Hokkaido University  Sapporo 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