Pemphigus Vegetans | Dermatology Education Pemphigus Vegetans Video
August 13, 2020

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Pemphigus Vegetans

Pemphigus Vegetans (PV) is a rare, variant form of pemphigus vulgaris. Clinically, patients develop vesicles, pustules, and bullae which rupture to form erosions with excessive granulation tissue and crusting. These may evolve into exudative, vegetating masses with pustules and fissures. Lesions tend to be located in the intertriginous areas, in the scalp, and on the face. Oral mucous membranes are almost always involved.

On histopathologic exam, one finds hyperkeratosis, papillomatosis, and acanthosis in addition to suprabasilar clefts with acantholytic cells and eosinophils. There is also typically a dense inflammatory infiltrate in the dermis composed of eosinphils, lymphocytes, and occasionally neutrophils. Direct immunofluorescence of perilesional skin demonstrates intercellular IgG and occasionally C3. Indirect immunofluorescence is positive for circulating IgG in the majority of patients.

The course is similar to that of pemphigus vulgaris. However, spontaneous remission is possible.