The relevance of complement in pemphigoid diseases: A critical appraisal

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Abstract

Pemphigoid diseases are autoimmune chronic inflammatory skin diseases, which are characterized by blistering of the skin and/or mucous membranes, and circulating and tissue-bound autoantibodies. The well-established pathomechanisms comprise autoantibodies targeting various structural proteins located at the dermal-epidermal junction, leading to complement factor binding and activation. Several effector cells are thus attracted and activated, which in turn inflict characteristic tissue damage and subepidermal blistering. Moreover, the detection of linear complement deposits in the skin is a diagnostic hallmark of all pemphigoid diseases. However, recent studies showed that blistering might also occur independently of complement. This review reassesses the importance of complement in pemphigoid diseases based on current research by contrasting and contextualizing data from in vitro, murine and human studies.

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Complement. Second of two parts.

M J Walport (2001)

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Complement. First of two parts.

Ian R. Mackay, Fred Rosen, Mark Walport (2001)

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Pemphigoid diseases.

Enno Schmidt, Detlef Zillikens (2013)

Pemphigoid diseases are a group of well defined autoimmune disorders that are characterised by autoantibodies against structural proteins of the dermal-epidermal junction and, clinically, by tense blisters and erosions on skin or mucous membranes close to the skin surface. The most common of these diseases is bullous pemphigoid, which mainly affects older people and the reported incidence of which in Europe has more than doubled in the past decade. Prognosis and treatments vary substantially between the different disorders and, since clinical criteria are usually not sufficient, direct immunofluorescence microscopy of a perilesional biopsy specimen or serological tests are needed for exact diagnosis. In eight pemphigoid diseases the target antigens have been identified molecularly, which has allowed the development of standard diagnostic assays for detection of serum autoantibodies-some of which are commercially available. In this Seminar we discuss the clinical range, diagnostic criteria, diagnostic assay systems, and treatment options for this group of diseases. Copyright © 2013 Elsevier Ltd. All rights reserved.

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Cristian Papara: URI : https://loop.frontiersin.org/people/1848892

Christian M. Karsten: URI : https://loop.frontiersin.org/people/481766

Hideyuki Ujiie: URI : https://loop.frontiersin.org/people/254022

Enno Schmidt: URI : https://loop.frontiersin.org/people/373973

Leon F. Schmidt-Jiménez: URI : https://loop.frontiersin.org/people/1038328

Adrian Baican: URI : https://loop.frontiersin.org/people/598281/overview

Patricia C. Freire: URI : https://loop.frontiersin.org/people/772691

Kentaro Izumi: URI : https://loop.frontiersin.org/people/506222

Katja Bieber: URI : https://loop.frontiersin.org/people/436423

Admar Verschoor: URI : https://loop.frontiersin.org/people/195197

Ralf J. Ludwig: URI : https://loop.frontiersin.org/people/23927

Jörg Köhl: URI : https://loop.frontiersin.org/people/25318

Detlef Zillikens: URI : https://loop.frontiersin.org/people/467981

Christoph M. Hammers: URI : https://loop.frontiersin.org/people/415606

Journal

Journal ID (nlm-ta): Front Immunol

Journal ID (iso-abbrev): Front Immunol

Journal ID (publisher-id): Front. Immunol.

Title: Frontiers in Immunology

Publisher: Frontiers Media S.A.

ISSN (Electronic): 1664-3224

Publication date (Electronic): 16 August 2022

Publication date Collection: 2022

Volume: 13

Electronic Location Identifier: 973702

Affiliations

[1] ¹ Department of Dermatology, University of Lübeck , Lübeck, Germany

[2] ² Department of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy , Cluj-Napoca, Romania

[3] ³ Institute of Systemic Inflammation Research, University of Lübeck , Lübeck, Germany

[4] ⁴ Department of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan

[5] ⁵ Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck , Lübeck, Germany

[6] ⁶ Division of Antibody-Based Immunotherapy, Department of Medicine II, Christian-Albrechts-University of Kiel and University Medical Center Schleswig-Holstein , Kiel, Germany

[7] ⁷ Department of Otorhinolaryngology, Klinikum rechts der Isar, Technical University Munich , Munich, Germany

[8] ⁸ Division of Immunobiology, Cincinnati Children’s Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati , OH, United States

Author notes

Edited by: Takashi Hashimoto, Osaka City University, Japan

Reviewed by: Jong Hoon Kim, Yonsei University, South Korea; Zhi Liu, University of North Carolina at Chapel Hill, United States; Ana Maria Abreu-Velez, Georgia Dermatopathology Associates (GDA), United States

*Correspondence: Christoph M. Hammers, christoph.hammers@ 123456uksh.de

This article was submitted to Autoimmune and Autoinflammatory Disorders, a section of the journal Frontiers in Immunology

Article

DOI: 10.3389/fimmu.2022.973702

PMC ID: 9434693

PubMed ID: 36059476

SO-VID: a55f1741-ccbd-479a-8be0-62c8631058b6

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This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

History

Date received : 20 June 2022

Date accepted : 26 July 2022

Page count

Figures: 2, Tables: 0, Equations: 0, References: 154, Pages: 13, Words: 5865

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