Dupilumab Combined with Glucocorticoids in the Treatment of Pemphigus Vegetans: Case Report.

Abstract

Introduction: Vegetative lesions arise from various causes, with typical examples including pemphigus vegetans and pyoderma vegetans (PDV). Differential diagnosis, treatment selection, and long-term management remain challenging. Systemic glucocorticoids are the conventional first-line therapy for pemphigus diseases, often combined with nonspecific immunosuppressants such as cyclosporine. Dupilumab, a monoclonal antibody targeting IL-4Rα, has recently been reported as a potential therapeutic option for pemphigus vegetans.

Case presentation: We report a 74-year-old woman presenting with painful perianal nodules, plaques, and ulcers for more than 3 months, accompanied by oral ulcers and weight loss. Skin biopsy demonstrated pseudoepitheliomatous hyperplasia with partial epidermal erosion. Laboratory testing revealed positive pemphigus antibodies, and skin secretion culture isolated Proteus mirabilis. Ambulatory electrocardiography indicated third-degree atrioventricular block. Positron emission tomography-computed tomography showed no evidence of malignancy. The patient was treated with dupilumab combined with systemic glucocorticoids and doxycycline, resulting in progressive shrinkage and eventual healing of the lesions.

Conclusion: In cases of vegetative lesions with pseudoepitheliomatous hyperplasia and positive Dsg3 antibodies, pemphigus vegetans should be considered, while PDV and paraneoplastic syndrome must be excluded. For patients with severe comorbidities, dupilumab combined with low-dose glucocorticoids may represent a therapeutic option after careful evaluation of immunosuppressive risks. To our knowledge, this is the first reported case of pemphigus vegetans successfully treated with dupilumab.