The clinical and histopathological spectrum of IgA-pemphigus--report of two cases

Clin Exp Dermatol. 1990 Nov;15(6):433-7. doi: 10.1111/j.1365-2230.1990.tb02138.x.

Abstract

Two new cases of neutrophilic bullous disease exhibiting bound and circulating intercellular IgA in vivo in both direct and indirect immunofluorescence (IF), for which the term IgA-pemphigus was recently proposed, are presented. The first case showed a unique constellation of clinical and histopathological features not previously described for IgA-pemphigus, resembling both dermatitis herpetiformis and pemphigus, for which the designation IgA-herpetiform pemphigus seems most appropriate. The second case showed clinical and histopathological features mimicking subcorneal pustular dermatosis for which the previously used term IgA-pemphigus foliaceus seems most appropriate. The previously reported 11 cases showing similar direct IF findings, as well as our two patients, illustrate the main differences between IgA-pemphigus and classic forms of this condition: (a) different clinical manifestations with the absence of Nikolsky's sign; (b) scanty acantholysis; an abundance of neutrophils, with occasional neutrophilic spongiosis; (c) absence of C3 on direct IF; (d) the lower sensitivity of indirect IF and low levels of circulating antibodies; (e) the relatively benign course of the disease; (f) the apparent responsiveness to sulphones in many cases. It is suggested that IgA pemphigus should be considered as an entity separate from, but closely related to, classic pemphigus.

Publication types

  • Case Reports

MeSH terms

  • Diagnosis, Differential
  • Female
  • Fluorescent Antibody Technique
  • Humans
  • Immunoglobulin A*
  • Middle Aged
  • Pemphigus / diagnosis
  • Pemphigus / metabolism
  • Pemphigus / pathology*
  • Skin / metabolism
  • Skin / pathology

Substances

  • Immunoglobulin A