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Bullous Pemphigoid Publisher Pubmed



Akbarialiabad H1, 2 ; Schmidt E3 ; Patsatsi A4 ; Lim YL5, 6, 7 ; Mosam A8 ; Tasanen K9, 10 ; Yamagami J11 ; Daneshpazhooh M12 ; De D13 ; Cardones ARG14 ; Joly P15 ; Murrell DF1, 2, 16
Authors
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Authors Affiliations
  1. 1. Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia
  2. 2. Australasian Blistering Diseases Foundation (ABDF), Sydney, NSW, Australia
  3. 3. Department of Dermatology and Lubeck Institute of Experimental Dermatology, University of Lubeck, Lubeck, Germany
  4. 4. Center of Expertise on AIBD, 2nd Dermatology Department, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece
  5. 5. National Skin Centre, Singapore, Singapore
  6. 6. Yong Loo Lin School of Medicine, Singapore, Singapore
  7. 7. Lee Kong Chian School of Medicine, Singapore, Singapore
  8. 8. Department of Dermatology, Inkosi Albert Luthuli Central Hospital and Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa
  9. 9. Department of Dermatology and Medical Research Center Oulu, Oulu University Hospital, Oulu, Finland
  10. 10. Research Unit of Clinical Medicine, Faculty of Medicine, University of Oulu, Oulu, Finland
  11. 11. Department of Dermatology, Tokyo Women’s Medical University, Tokyo, Japan
  12. 12. Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
  13. 13. Department of Dermatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
  14. 14. Division of Dermatology, Department of Internal Medicine, University of Kansas Medical Center, Lawrence, KS, United States
  15. 15. Dermatology Department, Rouen University Hospital, INSERM U1234, Normandie University, Rouen, France
  16. 16. Department of Dermatology, St George Hospital, Sydney, NSW, Australia

Source: Nature Reviews Disease Primers Published:2025


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. © Springer Nature Limited 2025.
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