Pemphigus Vegetans

Pemphigus is a group of vesiculobullous autoimmune diseases. The most common type of pemphigus is pemphigus vulgaris, characterized by mucocutaneous bullae. Among the many types of pemphigus, the rarest clinical variant is pemphigus vegetans. It distinguishes itself from pemphigus vulgaris by the formation of vegetative plaques in intertriginous areas and oral mucosa. Thought to be a subtype of pemphigus vulgaris, pemphigus vegetans represents 1-2% of all pemphigus . It is often difficult to diagnose and clinically misleading due to the variable presentation and presence of verrucous vegetations. There are two clinically recognized forms of pemphigus vegetans: the Hallopeau and Neumann. The Hallopeau type has an indolent course and characteristically demonstrates pustules that heal as vegetative plaques. The Neumann type is more severe and refractory to treatment, with vegetations that develop during an eruption of vesiculobullous lesions. The oral mucosa is usually involved. The most common cause of pemphigus vegetans is autoantibodies against desmoglein 3 (DSG3) and desmoglein 1 (DSG1). The mainstay of treatment for pemphigus vegetans is similar to that of pemphigus vulgaris, consisting of systemic corticosteroids and immunosuppressants.