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This paper systematically reviews the current articles regarding the use of dupilumab in the treatment of bullous pemphigoid (BP) to evaluate its safety and efficacy. PubMed, Embase, Cochrane Library, and Web of Science databases were searched for publications on dupilumab for BP from inception to 10 March, 2023. A total of 26 studies were included for systematic review. The primary outcome was clinical remission, and the secondary outcomes were recurrence and adverse events. Among 96 patients, 71.8% (n = 69/96) received systemic or topical steroids, immunosuppressants, immunomodulators, intravenous immunoglobulins, antihistamines, plasmapheresis, rituximab, and omalizumab, but none of them were successful. After dupilumab treatment, 66.7% (n = 64/96) of patients achieved complete remission, 25.0% (n = 24/96) had partial remission, 5.2% (n = 5/96) showed no remission, and no patients experienced deterioration. In addition, 1.0% (n = 1/96) and 2.0% (n = 2/96) patients stopped using dupilumab due to adverse reactions and cost, respectively. The average remission time was 4.5 months. 46.2% (n = 25/96) of the patients were followed up with a median follow-up of 8 months and only 2 patients relapsed at 8 and 7 months, respectively. Adverse event was 16.9% (n = 12/71), of which transient eosinophilia was the most common. This study indicates that the dupilumab is a promising treatment for BP with high clinical benefit associated with low recurrence rate, adverse event rate, and mortality. However, a large-scale randomized controlled trial is needed to further confirm the safety and efficacy of dupilumab in patients with BP treatment. [ABSTRACT FROM AUTHOR]

• Dupilumab, as an IL4Rα antagonist, showed a beneficial effect on BP. • We reported the clinical data for nine BP patients treated with dupilumab. • Previous literature describing the use of dupilumab for BP was summarized. Bullous pemphigoid is an autoimmune blistering disease that affects the elderly mostly. First-line treatment of systemic corticosteroids may cause significant adverse effects, especially in patients with multiple co-morbidities. Dupilumab shows certain effectiveness in treating BP. We aim to profile our experience with dupilumab in a series of patients with BP and review the articles published to date. Medical records of 9 patients with moderate-to-severe BP were retrospectively reviewed. All patients were administered dupilumab. Response to dupilumab was evaluated by NRS scores, number of lesions, and the systemic corticosteroids' dosage. The PubMed, Embase, and Web of Science databases were searched to identify eligible studies. The 9 patients were identified in this case series with a median age of 68 years (range 42–89) and the median duration of disease before being treated with dupilumab was 6 months (range 1–144). Complete remission was achieved in 6 patients while partial response was achieved in one patient. The NRS score had decreased to varying degrees at week 2 in all patients, and skin lesions improved within 2 to 6 weeks. Fifteen publications were included: 3 retrospective studies and 12 case series or reports, with a total of 63 patients. The overall complete response and partial response rates were 74.6 % and 11.1 %, respectively. Dupilumab appears to be a safe alternative for the treatment of patients with refractory BP. [ABSTRACT FROM AUTHOR]