Immunoglobulin M pemphigoid - 12/02/21

Doi : 10.1016/j.jaad.2021.01.017

Katharina Boch, MD ^a, Christoph M. Hammers, MD, PhD ^a,^b, Stephanie Goletz, PhD ^b, Mayumi Kamaguchi, PhD ^b, Ralf J. Ludwig, MD ^b, Stefan W. Schneider, MD ^c, Detlef Zillikens, MD ^a, Eva Hadaschik, MD ^d, Enno Schmidt, MD, PhD ^a,^b,^⁎
^a Department of Dermatology, University of Lübeck, Lübeck, Germany
^b Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany
^c Department of Dermatology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
^d Department of Dermatology, University of Essen, Essen, Germany

^∗Reprint requests: Enno Schmidt, MD, PhD, Lübeck Institute of Experimental Dermatology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.Lübeck Institute of Experimental DermatologyUniversity of LübeckRatzeburger Allee 160Lübeck23538Germany

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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.

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Key words : IgM, pemphigoid, pemphigoid diseases, subepidermal autoimmune blistering disease(s)

Abbreviations used : AIBD, C3, Col17, Col7, DEJ, DIF, IgA, IgG, IgM, IIF, WM

Plan

Methods

Patient and control sera

Hematoxylin–eosin stain

DIF and indirect immunofluorescence microscopy

Enzyme-linked immunosorbent assays

Expression and purification of recombinant Col17 fragments

Immunoblotting

Cryosection assay

Complement fixation test

Internalization assay

Results

Discussion

	Drs Boch, Hammers, and Goletz contributed equally to this article.
	Funding sources: Supported by structural funding from the Deutsche Forschungsgemeinschaft through CRU 303 Pemphigoid Diseases and Excellence Cluster 2167/1 Precision Medicine in Chronic Inflammation (to Drs Ludwig, Zillikens, and Schmidt).
	IRB approval status: Approved by the local IRB.