Atypical Clinical and Serological Manifestation of Pemphigus Vegetans: A Case Report and Review of the Literature

Abstract

Pemphigus vegetans (PVeg) is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. Exceptional manifestations may be misdiagnosed resulting in delayed diagnosis and treatment. Diagnosis is confirmed by immunofluorescence and detection of anti-desmoglein (Dsg) 3 and/or anti-Dsg1 antibodies. We herein report an unusual manifestation of PVeg. At the time of first presentation, lesions were restricted to the right ring finger's tip. Although mucous membranes were initially not affected, high levels of anti-Dsg3 antibodies were detected while anti-Dsg1 and anti-desmocollin (Dsc) 1, 2, and 3 antibodies were absent. To compare our immunological findings with previous reports, all accessible Anglophone literature published since December 1988 was evaluated. We identified 52 patients suffering from PVeg, 7 of these showed anti-Dsg3 antibodies without any mucous membrane involvement. Notably, the detection of anti-Dsg1 and anti-Dsg3 antibodies does not necessarily correlate with the involvement of skin and/or mucous membranes. This might be due to more specific and complex antibody constellations in nonclassical or atypical pemphigus.

Keywords: Desmocollin; Desmoglein; Pemphigus; Pemphigus vegetans; Review.

Fig. 1

a Plaque with overlying crusts and annular pustules on erythematous ground on the fourth right digit. b Histopathology revealed prominent acanthosis with an intraepidermal eosinophilic pustule accompanied by surrounding eosinophilic spongiosis and a superficial eosinophil-rich inflammatory infiltrate and acantholysis. c Direct IF microscopy of perilesional skin showing intercellular deposits of IgG antibodies in the epidermis. IF, immunofluorescence.

Fig. 2

Serum autoantibody levels during the course of the disease. Black dots indicate the level of anti-Dsg3 antibodies. White squares indicate the level of anti-Dsg1 antibodies. Adjuvant treatment consisted of azathioprine or mycophenolate mofetil. Each solid-lined black arrow indicates a steroid pulse (100 mg dexamethasone on 3 consecutive days). The dashed arrows refer to rituximab administration (1 g). Disease activity is indicated by gray arrows. AZA, adjuvant treatment consisted of azathioprine.