Nine cases of refractory bullous pemphigoid treated with dupilumab and literature review

Abstract

Background and aims: 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.

Methods: 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.

Results: 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.

Conclusion: Dupilumab appears to be a safe alternative for the treatment of patients with refractory BP.

Keywords: Bullous pemphigoid; Dupilumab; Interleukin 13; Interleukin 4; Treatment.