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

Abstract

Benign familial pemphigus (BFP) is a chronic autosomal dominant genodermatosis characterized by the appearance of flaccid blisters that progress to painful erythematous macerated plaques and erosions in intertriginous areas. Although various treatments for BFP exist, current treatments are not consistently effective, and BFP symptoms often cause a decreased quality of life. We report a case series of three patients with refractory moderate-to-severe BFP who were successfully treated with dupilumab. We also describe their clinical evolution over a follow-up period of 18 months. All patients reported a decrease in pruritus and improved skin findings, with no adverse side-effects. However, increased dosing was required in one patient to achieve optimal control of the disease. We also discuss previously reported cases of BFP treated with biologics. Dupilumab appears to be a useful and safe treatment option for patients with refractory BFP.