Clinical Characterization and Outcomes of Dupilumab for Pemphigoid Gestationis

Treatment of pemphigoid gestationis (PG) mainly consists of systemic and potent topical corticosteroids. This retrospective case series describes patients with severe PG treated with dupilumab, including serological follow-up, and reviews all published cases. PG was confirmed by direct immunofluorescence (linear C3 and/or IgG deposition along the basement membrane zone) and serology (IgG epidermal staining on salt-split skin or anti-BP180 NC16A IgG ELISA). Pruritus, quality of life and anti-BP180 NC16A IgG and anti-BP180 NC16A IgE were monitored longitudinally. In 5 pregnant women, dupilumab was initiated with a 600 mg loading dose followed by 300 mg biweekly. Baseline pruritus was severe (NRS 8-10), with high DLQI scores (18-30). Blistering ceased within 1 week in 2 patients and within 4 weeks in 1. Two patients required weekly dosing for disease control. Systemic corticosteroids were discontinued in 3 patients and tapered to ≤10 mg/day in 2 before delivery. In one case, anti-BP180 IgG paralleled clinical improvement. Anti-BP180 IgE remained negative. All patients delivered healthy infants. In 10 reported cases, pruritus improved rapidly and systemic corticosteroids were tapered. These findings suggest dupilumab may be a promising corticosteroid-sparing option for PG, with rapid clinical response and a favourable safety profile, despite off-label use in pregnancy.

Abstract from PubMed