Pemphigus vegetans with paronychia‐like changes resistant to rituximab therapy

Abstract

Pemphigus vegetans (PVeg) is a clinical variant of pemphigus vulgaris (PV) that makes up about 2% of all cases. It is distinguished from PV by the presence of vegetative plaques that are usually found in the oral mucosa or intertriginous areas. PVeg can present as one of two clinical subtypes: Hallopeau type and Neumann type. The Hallopeau type is a milder form of the disease, often sparing the oral mucosa. The Neumann type is a more severe form that often includes oral mucosal involvement. Nail unit disease of PVeg is rare, lending to limited recommendations for management. Herein, we present a case of PVeg with paronychia-like changes following rituximab therapy, along with a brief review of PVeg nail unit disease management. Previously reported treatments include prednisone, azathioprine, dapsone, and cyclosporine. In this patient with nail disease, despite rituximab therapy, intralesional triamcinolone acetonide was effective, thereby avoiding the need for additional immunosuppression.