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The disease is characterized primarily by autoantibodies to hemidesmosomal proteins BP180 and\/or BP230, and an inflammatory reaction with notable features of type\u00a02 inflammation, including elevated serum IgE, increased numbers of eosinophils in lesions and peripheral blood, and elevated expression of type\u00a02 cytokines and chemokines in skin lesions. In this review, we present what is known about BP pathophysiology, including the role of type\u00a02 inflammation, and discuss how findings from studies of biologics targeting type\u00a02 immune mediators have helped to clarify the biological mechanisms driving BP pathophysiology. Future studies of these targeted therapies and others in development will help to further elucidate the mechanisms underlying BP pathophysiology and potentially provide better treatment options for patients.<\/jats:p>","DOI":"10.1007\/s12325-024-02992-w","type":"journal-article","created":{"date-parts":[[2024,10,19]],"date-time":"2024-10-19T06:02:10Z","timestamp":1729317730000},"page":"4418-4432","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Pathophysiology of Bullous Pemphigoid: Role of Type\u00a02 Inflammation and Emerging Treatment Strategies (Narrative Review)"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3030-5369","authenticated-orcid":false,"given":"Victoria P.","family":"Werth","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D\u00e9d\u00e9e F.","family":"Murrell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Joly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renata","family":"Heck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie M.","family":"Orengo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marius","family":"Ardeleanu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Verena","family":"Hultsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,10,19]]},"reference":[{"key":"2992_CR1","doi-asserted-by":"publisher","first-page":"1998","DOI":"10.1038\/jid.2012.35","volume":"132","author":"P Joly","year":"2012","unstructured":"Joly P, Baricault S, Sparsa A, et al. 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Werth has consulted for AbbVie, Argenx, AstraZeneca, Janssen, Kyowa Hakko Kirin, Lilly, Principia Biopharma, Regeneron Pharmaceuticals Inc., and Roche-Genentech; reports grants from Argenx, Roche-Genentech, Sanofi-Regeneron Pharmaceuticals Inc., and Syntimmune; and is a co-creator of BPDAI. D\u00e9d\u00e9e F. Murrell has served as an investigator and on the advisory board for Argenx, Principia Biopharma, Regeneron Pharmaceuticals Inc., and Roche, and as an investigator for AstraZeneca; and is a co-creator of BPDAI and creator of ABQOL. Pascal Joly reports consultancy for Amgen, Argenx, AstraZeneca, Innovaderm Research, Principia Biopharma, Roche, Sanofi-Regeneron Pharmaceuticals Inc., and Thermo Fisher Scientific. Renata Heck has served as an investigator for Amgen and Pfizer. Jamie M. Orengo and Marius Ardeleanu are employees of Regeneron Pharmaceuticals Inc., and may hold company stock and or\/stock options. Jamie M. Orengo is an officer of Regeneron Pharmaceuticals Inc. Verena Hultsch is an employee of Sanofi and may hold stock and\/or stock options in the company.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}},{"value":"This narrative review article is based on previously conducted studies and does not contain any new studies with human participants or animals performed by any of the authors.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}}]}}