The Neumann Type of Pemphigus Vegetans Treated with Combination of Dapsone and Steroid

Abstract

Pemphigus vegetans is a rare variant of pemphigus vulgaris and is characterized by vegetating lesions in the inguinal folds and mouth and by the presence of autoantibodies against desmoglein 3. Two clinical subtypes of pemphigus vegetans exist, which are initially characterized by flaccid bullae and erosions (the Neumann subtype) or pustules (the Hallopeau subtype). Both subtypes subsequently develop into hyperpigmented vegetative plaques with pustules and hypertrophic granulation tissue at the periphery of the lesions. Oral administration of corticosteroids alone does not always induce disease remission in patients with pemphigus vegetans. We report here on a 63-year-old woman with pemphigs vegetans. She had a 2-year history of vegetating, papillomatous plaques on the inguinal folds and erosions of the oral mucosa. The enzyme-linked immunosorbent assay was positive for anti-desmoglein 3, but it was negative for anti-desmoglein 1. She was initially treated with systemic steroid, but no improvement was observed. The patient was then successfully treated with a combination of systemic steroid and dapsone with a good clinical response.

Pemphigus vegetans with coexistent herpes simplex infection and deep venous thrombosis of the lower extremities

“…Research has shown that agents such as azathioprine, mycophenolate mofetil and dapsone may improve the rate of remission in these patients 1 4. Recently, rituximab therapy was shown to be useful in treating recalcitrant PVeg.…”

“…Pemphigus vegetans (PVeg) is the rarest clinical variant of PV, accounting for 1–2% of all pemphigus. It is characterised by vegetating plaques primarily localised to the flexural areas 1. Its management is similar to that of PV.…”

Cobblestone mouth: An exuberant oral presentation of pemphigus vegetans

“…Pyodermatitispyostomatitis vegetans can clinically and histopathologically resemble pemphigus vegetans and only indirect and direct immunofluorescence studies can accurately distinguish between these two entities. 6 …”

Cobblestone mouth: An exuberant oral presentation of pemphigus vegetans

“…6 Pyodermatitispyostomatitis vegetans can clinically and histopathologically resemble pemphigus vegetans and only indirect and direct immunofluorescence studies can accurately distinguish between these two entities.…”