TY - JOUR
T1 - Immunoglobulin M pemphigoid
AU - Boch, Katharina
AU - Hammers, Christoph M.
AU - Goletz, Stephanie
AU - Kamaguchi, Mayumi
AU - Ludwig, Ralf J.
AU - Schneider, Stefan W.
AU - Zillikens, Detlef
AU - Hadaschik, Eva
AU - Schmidt, Enno
N1 - Publisher Copyright:
© 2021 American Academy of Dermatology, Inc.
Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.
PY - 2021
Y1 - 2021
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85100805614&partnerID=8YFLogxK
U2 - 10.1016/j.jaad.2021.01.017
DO - 10.1016/j.jaad.2021.01.017
M3 - Journal articles
C2 - 33453342
AN - SCOPUS:85100805614
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
SN - 0190-9622
ER -