Immunoglobulin M pemphigoid

Katharina Boch, Christoph M. Hammers, Stephanie Goletz, Mayumi Kamaguchi, Ralf J. Ludwig, Stefan W. Schneider, Detlef Zillikens, Eva Hadaschik, Enno Schmidt*

*Corresponding author for this work

Abstract

Background: Pemphigoid diseases are a heterogeneous group of autoimmune blistering disorders characterized by predominant deposition of immunoglobulin G or immunoglobulin A autoantibodies against structural proteins of the dermoepidermal junction (DEJ). Sole linear immunoglobulin M (IgM) deposits at the DEJ in pemphigoid diseases have been observed; however, IgM-specific target antigens have not been identified. Objective: Characterization of patients with IgM pemphigoid. Methods: Skin biopsy specimens and sera from IgM-positive patients were assessed using histopathology, direct and indirect immunofluorescence microscopy, enzyme-linked immunosorbent assays, immunoblotting, cryosection assay, complement fixation test, and internalization assays. Results: Tissue-bound linear IgM deposits along the DEJ and circulating IgM autoantibodies against type XVII collagen (Col17) were detected. These circulating IgM autoantibodies showed no complement activating or blister inducing capacity, but the ability of Col17 internalization ex vivo. Limitations: Limited number of patients. Conclusion: This study provides further evidence for the role of IgM autoantibodies in pemphigoid disease and highlights Col17 as a target antigen in IgM pemphigoid.

Original languageEnglish
JournalJournal of the American Academy of Dermatology
Volume85
Issue number6
Pages (from-to)1486-1492
Number of pages7
ISSN0190-9622
DOIs
Publication statusPublished - 12.2021

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)
  • Centers: Center for Research on Inflammation of the Skin (CRIS)

DFG Research Classification Scheme

  • 2.21-05 Immunology
  • 2.22-19 Dermatology

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