Diagnosis of autoimmune subepidermal bullous diseases with mucous membrane involvement based on laser-scanning confocal microscopy

Eur J Dermatol. 2020 Oct 1;30(5):516-523. doi: 10.1684/ejd.2020.3765.

Abstract

Background: Mucosal involvement in autoimmune subepidermal blistering disorders (ASBD) may represent the only or predominant localization. Circulating autoantibodies are detected in 50% cases.

Objective: The aim of this study was to evaluate the usefulness of fluorescence overlay antigen mapping by laser-scanning confocal microscopy (FOAM-LSCM) to identify ASBD with mucosal involvement in oral mucosa specimens.

Materials & methods: Thirty-two ASBD patients, diagnosed based on direct immunofluorescence between 2006 and 2016, were enrolled. Localization of IgG deposits bound at the basement membrane zone, relative to laminin-332 and collagen IV localization, was assessed in vivo.

Results: FOAM-LSCM disclosed four different immunofluorescence patterns. IgG deposits were located above laminin-332, as in bullous pemphigoid (BP-type), in 19% cases and co-localized with laminin-332 (anti-laminin-332-type) in 6% cases. IgG deposits were found below laminin-332 and above collagen IV (mucous membrane pemphigoid-type) in 59% cases, and below collagen IV (epidermolysis bullosa acquisita-type) in 16%. Circulating antibodies were found in 56% cases.

Conclusion: The FOAM-LSCM method should be used in order to obtain a definitive diagnosis of ASBD with mucosal involvement, particularly in the presence of negative circulating antibodies.

Keywords: autoimmune subepidermal bullous diseases; diagnostics; fluorescence overlay antigen mapping by laser-scanning confocal microscopy; mucous membrane pemphigoid.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Autoantibodies / blood
  • Basement Membrane / immunology
  • Cell Adhesion Molecules / immunology
  • Collagen Type IV / immunology
  • Epidermolysis Bullosa Acquisita / diagnostic imaging*
  • Epidermolysis Bullosa Acquisita / immunology*
  • Female
  • Fluorescent Antibody Technique, Direct*
  • Humans
  • Immunoglobulin G / analysis
  • Kalinin
  • Male
  • Microscopy, Confocal*
  • Middle Aged
  • Mouth Mucosa / diagnostic imaging*
  • Mouth Mucosa / immunology*

Substances

  • Autoantibodies
  • Cell Adhesion Molecules
  • Collagen Type IV
  • Immunoglobulin G