Pemphigus vegetans (PVeg), a rare variant of pemphigus vulgaris (PV), is an autoimmune bullous disease characterized by vegetating lesions in intertriginous areas. Circulating immunoglobulin G (IgG) autoantibodies against desmoglein 3 (with or without desmoglein 1, desmocollin 3), a keratinocyte cell surface molecule, lead to disruption of normal cell adhesion between keratinocytes and subsequent formation of vesicles and bullae.

PVeg is rare and makes up an estimated 2%-5% of the total cases of pemphigus. Although this disease can affect people of all age groups, it occurs primarily during middle age with a median onset of 40-60 years. It affects males and females equally. New onset and flares of PVeg after SARS-CoV-2 vaccination have been reported.

PVeg has been divided into 2 subtypes depending on clinical presentation and disease course:

• The Neumann type is characterized by flaccid bullae and erosions in intertriginous areas that develop overlying vegetations. This subtype is more common, with a clinical course that parallels PV.
• The Hallopeau type has a more benign course and is characterized by erupting crops of pustules that evolve into crusted plaques. It may remit spontaneously.

Mucous membrane involvement is a frequent occurrence. The scalp may also manifest vegetative plaques. The perioral area is a site of predilection in the Hallopeau type.