The role of Dupilumab in the treatment of Bullous Pemphigoid, Pemphigus and Hailey-Hailey Disease: A Narrative Review
DOI:
https://doi.org/10.12775/JEHS.2026.91.70847Keywords
Dupilumab, Bullous pemphigoid, Pemphigus vulgaris, Pemphigus foliaceus, Hailey-Hailey disease, Autoimmune bullous diseasesAbstract
Background: Autoimmune bullous diseases (AIBDs) and Hailey-Hailey disease are chronic skin disorders characterized by blister formation, pruritus, and reduced quality of life. Standard treatments, including systemic corticosteroids and immunosuppressants, often cause adverse effects and may not achieve long-term remission. Dupilumab, a monoclonal antibody targeting IL-4 and IL-13, has emerged as a promising alternative.
Aim: To evaluate the efficacy and safety of dupilumab in patients with bullous pemphigoid, pemphigus vulgaris and foliaceus, and Hailey-Hailey disease.
Material and Methods: Literature review including meta-analyses, randomized trials, retrospective studies, and case reports on dupilumab use in AIBDs and Hailey-Hailey disease. Disease activity was assessed using PDAI, BPDAI, or clinical evaluation; quality of life and adverse events were summarized.
Results: In bullous pemphigoid, dupilumab reduced disease activity, pruritus, and cumulative corticosteroid dose, with most patients achieving complete or partial remission. Evidence in pemphigus vulgaris and foliaceus is limited but suggests potential benefit, particularly in patients ineligible for rituximab. In Hailey-Hailey disease, dupilumab improved lesions and quality of life, although relapse after discontinuation was reported. Therapy was generally well tolerated- with main adverse events being: injection site reactions, conjunctivitis, and eosinophilia.
Conclusions: Dupilumab is an effective and safe option in bullous pemphigoid, offering pruritus relief and steroid-sparing benefits. Its use in pemphigus and Hailey-Hailey disease is promising but experimental. Further high-quality studies are needed to confirm long-term efficacy and safety.
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Copyright (c) 2026 Natalia Będkowska-Kuśmierek, Sabina Zaborowska, Paulina Pawlak , Olaf Wojcieszuk , Łukasz Starczewski, Adrianna Babik, Kamila Kamińska , Anna Złotnik, Matylda Będkowska-Kuśmierek, Kinga Krzysztofik
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