Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience

An Bras Dermatol. 2014 Nov-Dec;89(6):885-9. doi: 10.1590/abd1806-4841.20143221.

Abstract

Background: Immunofluorescence testing is an important tool for diagnosing blistering diseases.

Objective: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases.

Methods: We retrospectively analyzed immunofluorescence results encompassing a 10-year period.

Results: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate.

Conclusion: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect immunofluorescence when Salt-split skin technique is performed.

MeSH terms

  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / immunology
  • Female
  • Fluorescent Antibody Technique / methods*
  • Humans
  • Immunoglobulin A / immunology
  • Immunoglobulin G / immunology
  • Male
  • Reproducibility of Results
  • Retrospective Studies
  • Sensitivity and Specificity
  • Skin Diseases, Vesiculobullous / diagnosis*
  • Skin Diseases, Vesiculobullous / immunology
  • Skin Tests

Substances

  • Immunoglobulin A
  • Immunoglobulin G