PEMPHIGUS VEGETANS


Pemphigus vegetans, which accounts for 1 to 2% of pemphigus cases, is a relatively benign variant of pemphigus vulgaris because the patient demonstrates the ability to heal the denuded areas. Two forms of pemphigus vegetans are recognized: the Neumann type and the Hallopeau type. The Neumann type is more common, and the early lesions are similar to those seen in pemphigus vulgaris, with large bullae and denuded areas. These areas attempt healing by developing vegetations of hyperplastic granulation tissue. In the Hallopeau type, which is less aggressive, pustules, not bullae, are the initial lesions. These pustules are followed by verrucous hyperkeratotic vegetations.

Biopsy results of the early lesions of pemphigus vegetans show suprabasilar acantholysis.In older lesions, hyperkeratosis and pseudoepitheliomatous hyperplasia become prominent. Immunofluorescent study shows changes identical to those seen in PV.
Oral Manifestations. Oral lesions are common in both forms of pemphigus vegetans and may be the initial sign of disease.Gingival lesions may be lace-like ulcers with a purulent surface on a red base or have a granular or cobblestone appearance (Figure 4-29). Oral lesions that are associated with inflammatory bowel disease and resemble pemphigus vegetans both clinically and histologically are referred to by some authors as pyostomatitis vegetans.

Treatment. Treatment is the same as that for PV.