D1.461 - Dupilumab as a Steroid-Sparing Treatment Option for Pemphigus Vulgaris: A Case Series from a Tertiary Centre

Background:Pemphigus vulgaris (PV) is a rare autoimmune blistering disease mediated by IgG autoantibodies against desmoglein 1 and 3, resulting in acantholysis and mucocutaneous blistering. Standard therapy relies on systemic corticosteroids and rituximab. Increasing evidence supports a role for Th2-driven pathways in PV pathogenesis, with interleukin-4 (IL-4) contributing to autoantibody production (Tavakolpour & Tavakolpour, Cytokine 2015). Dupilumab, an IL-4 receptor alpha antagonist, inhibits IL-4/IL-13 signalling and has been reported in emerging case literature as a potential adjunctive therapy (Jiang et al., JAAD Case Rep 2023; Moore & Hurley, JAAD Case Rep 2022).

Methods:We report four patients with biopsy- and immunofluorescence-confirmed PV treated with dupilumab at a tertiary centre. Clinical response, steroid requirements, and safety were assessed longitudinally.

Results:Four female patients (45–68 years) with mucosal or mucocutaneous PV received dupilumab (600 mg loading dose followed by 300 mg every two weeks) alongside steroid-sparing immunosuppressants.

Clinical improvement was observed within 7–12 weeks in all cases. One patient achieved complete resolution of oral and pharyngeal lesions by 13 weeks with sustained remission at 23 weeks. Another demonstrated marked improvement by 12 weeks with successful corticosteroid tapering. A third patient achieved 70–80% reduction in disease activity by 36 weeks with reduced flare frequency. The fourth discontinued systemic steroids by 13 weeks and remained clinically stable at 47 weeks.

Dupilumab was well tolerated, with no severe flare-ups or serious adverse events reported.

Conclusions:In this case series, dupilumab demonstrated favourable safety and meaningful steroid-sparing benefit in refractory PV. Targeting IL-4/IL-13 signalling may represent a promising adjunctive strategy in selected patients. Larger controlled studies are required to define efficacy and optimal positioning within PV treatment algorithms.