Refractory benign familial pemphigus successfully treated with dupilumab: a case series.

Abstract

Benign familial pemphigus (BFP) is a chronic autosomal dominant dermatosis characterized by the appearance of flaccid blisters which evolve to painful erythematous macerated plaques and erosions in intertriginous areas. While different medical therapies for BFP exist, current treatments are not consistently effective, and symptoms often cause decreased quality of life. We report a case series of three patients with refractory moderate-to severe BFP which have been successfully treated with dupilumab, describing clinical evolution within a follow-up period of 18 months. All patients reported a decrease in pruritus and improved skin findings with no adverse effects; although in one patient, increased dosing was required to achieve optimal control of disease. We also discuss previous reported cases of BFP treated with biologics. Dupilumab seems to be a useful and safe therapeutic option for refractory BFP cases.