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Review
. 2025 Feb 20;11(1):12.
doi: 10.1038/s41572-025-00595-5.

Bullous pemphigoid

Hossein Akbarialiabad #  1   2 , Enno Schmidt #  3 , Aikaterini Patsatsi  4 , Yen Loo Lim  5   6   7 , Anisa Mosam  8 , Kaisa Tasanen  9   10 , Jun Yamagami  11 , Maryam Daneshpazhooh  12 , Dipankar De  13 , Adela Rambi G Cardones  14 , Pascal Joly #  15 , Dedee F Murrell #  16   17   18
Affiliations
Review

Bullous pemphigoid

Hossein Akbarialiabad et al. Nat Rev Dis Primers. .

Erratum in

  • Author Correction: Bullous pemphigoid.
    Akbarialiabad H, Schmidt E, Patsatsi A, Lim YL, Mosam A, Tasanen K, Yamagami J, Daneshpazhooh M, De D, Cardones ARG, Joly P, Murrell DF. Akbarialiabad H, et al. Nat Rev Dis Primers. 2025 Mar 3;11(1):16. doi: 10.1038/s41572-025-00605-6. Nat Rev Dis Primers. 2025. PMID: 40032865 No abstract available.

Abstract

Bullous pemphigoid is a chronic, subepidermal autoimmune blistering disease characterized by tense blisters on erythematous or normal skin that predominantly affects the older population. The disease arises from autoantibodies targeting hemidesmosomal proteins BP180 and BP230, which are crucial for dermal-epidermal adhesion. The incidence of bullous pemphigoid is increasing, attributed to an ageing population and improved diagnostic recognition. Genetic predisposition, environmental triggers and associations with other autoimmune disorders underline its multifactorial nature. Diagnosis involves clinical presentation, histopathology, direct immunofluorescence and serological tests. Treatment aims to reduce symptoms and prevent new blister formation, using corticosteroids, immunosuppressive agents and biologics such as rituximab and omalizumab. Despite therapeutic advancements, challenges persist in long-term management, especially in older patients with comorbidities. Ongoing research into molecular mechanisms and novel therapeutic targets and clinical trials are crucial for the development of safer and more effective treatments.

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Conflict of interest statement

Competing interests: H.A. is funded by the Australasian Blistering Diseases Foundation (ABDF). E.S. has a scientific cooperation with Euroimmun and holds patents with them that are not related to BP. E.S. also has scientific cooperations with Biotest, ArgenX, Fresenius Medical Care, AstraZeneca, Dompe and Sanofi, and has received honoraria from ArgenX, Fresenius Medical Care, Sanofi, Leo, Chugai and Almirall. A.P. has served as an investigator, advisory board member and speaker for AbbVie, Amgen, ArgenX, AstraZeneca, Jannsen, GenesisPharma SA, GSK, Leo, Νοvartis, Pfizer, Sanofi, Lilly and UCB. Y.L.L. is Secretary-General for the World Congress of Dermatology (WCD) 2023 in Singapore and a member of the Scientific Program Committee for the WCD2023, ILDS. She is also on the ad hoc advisory board for Sanofi at WCD2023 (honorarium paid to National Skin Centre, Singapore.). K.T. received an educational grant from Novartis and honoraria from Takeda. J.Y. is a consultant for Zenyaku Kogyo and Ono Pharma. D.D. is secretary of the International Pemphigus and Pemphigoid Conference, Chandigarh (2024). He is investigator for ArgenX (Pemphigus trial, now completed). A.R.G.C. is an author for UpToDate (with honorarium) and site principal investigator for Pfizer and Daiichi Sankyo, both for phase II studies related to lupus. She will be paid an honorarium by Elsevier for a review (DRESS syndrome). She is also secretary-treasurer of the Medical Dermatology Society, on the board of directors for the Society for Dermatology Hospitalists and is a Member of the Scientific Committee for the Society for Investigative Dermatology. P.J. is consultant for Amgen, ArgenX, AstraZeneca, Thermofisher, Sanofi, Akari, Janssen, Novartis, Servier, Chugai, Kezar Life Science, Regeneron, UCB, Principabio, Zenyaku Kogyo, Dualyx, Amgen and JJP Biologics. D.F.M. is an author for UptoDate for BP, has scientific cooperations and advisory roles in the field of eczema with Sanofi, AbbVie, Arena, Attovia, Incyte, Leo, Lilly, Galderma, Dermira, Pfizer, BMS, Kineska, Fujisawa, Novartis and Evelo. In the area of blistering diseases, she has scientific cooperations and advisory roles with ArgenX, Sanofi Regeneron, Principia, Janssen, Lilly, Ono, Almirall, RAPT and Roche. She is creator of the ABQOL/TABQOL and co-creator of the BPDAI. A.M. and M.D. declare no competing interests.

References

    1. Persson, M. S. et al. The global incidence of bullous pemphigoid: a systematic review and meta‐analysis. Br. J. Dermatol. 186, 414–425 (2022). This systematic review and meta-analysis maps the global distribution of BP. - PubMed - DOI
    1. Schmidt, E. & Zillikens, D. Pemphigoid diseases. Lancet 381, 320–332 (2013). - PubMed - DOI
    1. Egami, S., Yamagami, J. & Amagai, M. Autoimmune bullous skin diseases, pemphigus and pemphigoid. J. Allergy Clin. Immunol. 145, 1031–1047 (2020). - PubMed - DOI
    1. Ständer, S., Schmidt, E., Zillikens, D., Ludwig, R. J. & Kridin, K. Immunological features and factors associated with mucocutaneous bullous pemphigoid–a retrospective cohort study. J. Dtsch Dermatol. Ges. 19, 1289–1295 (2021). - PubMed
    1. Kridin, K. & Bergman, R. Assessment of the prevalence of mucosal involvement in bullous pemphigoid. JAMA Dermatol. 155, 166–171 (2019). - PubMed - PMC - DOI

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