Pemphigus vegetans (PVeg), a rare variant of pemphigus vulgaris (PV), is an autoimmune bullous disease characterized by vegetating lesions in intertriginous areas. As in PV, circulating immunoglobulin G (IgG) autoantibodies against desmoglein, a keratinocyte cell surface molecule, lead to disruption of normal cell adhesion between keratinocytes and subsequent formation of vesicles and bullae. Desmocollins have been noted to be additional autoantibody targets in PVeg.

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.

PVeg has been divided into two 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.