Atypical pemphigus: discordance between clinicopathological findings and the antigenic profile in four cases

J Cutan Pathol. 2006 Jul;33(7):502-7. doi: 10.1111/j.1600-0560.2006.00478.x.

Abstract

Background: The diagnosis of pemphigus vulgaris and pemphigus foliaceus is usually based on clinical, histological, and immunofluorescence (IF) findings. In recent years, the antigenic profile of both diseases has been further defined by immunobiochemical techniques (ELISA, immunoblot, and immunoprecipitation).

Methods: A retrospective study of 40 pemphigus patients was performed to determine the clinical, histological, and antigenic profile in patients with pemphigus followed at our Department. Charts review, clinical data, histological and IF findings, and antigenic analysis by ELISA were performed in all patients.

Results: In most patients, there was a perfect correlation between the clinical and histological findings and their antigenic profile. In four patients (10%), clinicopathological features and antigenic findings were discordant.

Conclusions: The antigenic profiles in pemphigus do not always correlate with the clinical diagnosis. Therefore, clinical and histological features should be considered as the mainstay for the diagnosis of pemphigus.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Autoantibodies / blood
  • Autoantigens / immunology
  • Desmoglein 1 / blood*
  • Enzyme-Linked Immunosorbent Assay
  • Epidermis / immunology
  • Epidermis / pathology*
  • Female
  • Fluorescent Antibody Technique, Direct
  • Fluorescent Antibody Technique, Indirect
  • Humans
  • Male
  • Middle Aged
  • Pemphigus / immunology
  • Pemphigus / pathology*
  • Retrospective Studies

Substances

  • Autoantibodies
  • Autoantigens
  • DSG1 protein, human
  • Desmoglein 1