The relevance of complement in pemphigoid diseases: A critical appraisal

Cristian Papara; Christian M. Karsten; Hideyuki Ujiie; Enno Schmidt; Leon F. Schmidt-Jiménez; Adrian Baican; Patricia C. Freire; Kentaro Izumi; Katja Bieber; Matthias Peipp; Admar Verschoor; Ralf J. Ludwig; Jörg Köhl; Detlef Zillikens; Christoph M. Hammers

doi:10.3389/fimmu.2022.973702

The relevance of complement in pemphigoid diseases: A critical appraisal

Cristian Papara, Christian M. Karsten, Hideyuki Ujiie, Enno Schmidt, Leon F. Schmidt-Jiménez, Adrian Baican, Patricia C. Freire, Kentaro Izumi, Katja Bieber, Matthias Peipp, Admar Verschoor, Ralf J. Ludwig, Jörg Köhl, Detlef Zillikens, Christoph M. Hammers^*

^*Corresponding author for this work

21 Citations (Scopus)

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.

Original language	English
Article number	973702
Journal	Frontiers in Immunology
Volume	13
ISSN	1664-3224
DOIs	https://doi.org/10.3389/fimmu.2022.973702
Publication status	Published - 16.08.2022

Funding

This research was supported by DFG funding for the Collaborative Research Center (CRC) 1526 – Pathomechanisms of Antibody-mediated Autoimmunity (PANTAU) – Insights from Pemphigoid Diseases (Grant No.: 454193335), the CRU 303 P7, the Research Training Group “Autoimmune Pre-Disease” (GRK 2633), the Schleswig-Holstein Excellence-Chair Program from the State of Schleswig Holstein, the Excellence Cluster EXC 2167 Precision Medicine in Chronic Inflammation (TI-4), and the Sinergia Unravel principles of self-organization in injured tissue (CRSII5_202301/1) from the Swiss National Science Foundation.

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

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

Access to Document

10.3389/fimmu.2022.973702

Cite this

Papara, C., Karsten, C. M., Ujiie, H., Schmidt, E., Schmidt-Jiménez, L. F., Baican, A., Freire, P. C., Izumi, K., Bieber, K., Peipp, M., Verschoor, A., Ludwig, R. J., Köhl, J., Zillikens, D., & Hammers, C. M. (2022). The relevance of complement in pemphigoid diseases: A critical appraisal. Frontiers in Immunology, 13, Article 973702. https://doi.org/10.3389/fimmu.2022.973702

@article{f833bc1499594586984c899ecfda400c,

title = "The relevance of complement in pemphigoid diseases: A critical appraisal",

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.",

author = "Cristian Papara and Karsten, \{Christian M.\} and Hideyuki Ujiie and Enno Schmidt and Schmidt-Jim{\'e}nez, \{Leon F.\} and Adrian Baican and Freire, \{Patricia C.\} and Kentaro Izumi and Katja Bieber and Matthias Peipp and Admar Verschoor and Ludwig, \{Ralf J.\} and J{\"o}rg K{\"o}hl and Detlef Zillikens and Hammers, \{Christoph M.\}",

note = "Publisher Copyright: Copyright {\textcopyright} 2022 Papara, Karsten, Ujiie, Schmidt, Schmidt-Jim{\'e}nez, Baican, Freire, Izumi, Bieber, Peipp, Verschoor, Ludwig, K{\"o}hl, Zillikens and Hammers.",

year = "2022",

month = aug,

day = "16",

doi = "10.3389/fimmu.2022.973702",

language = "English",

volume = "13",

journal = "Frontiers in Immunology",

issn = "1664-3224",

publisher = "Frontiers Media",

}

TY - JOUR

T1 - The relevance of complement in pemphigoid diseases

T2 - A critical appraisal

AU - Papara, Cristian

AU - Karsten, Christian M.

AU - Ujiie, Hideyuki

AU - Schmidt, Enno

AU - Schmidt-Jiménez, Leon F.

AU - Baican, Adrian

AU - Freire, Patricia C.

AU - Izumi, Kentaro

AU - Bieber, Katja

AU - Peipp, Matthias

AU - Verschoor, Admar

AU - Ludwig, Ralf J.

AU - Köhl, Jörg

AU - Zillikens, Detlef

AU - Hammers, Christoph M.

PY - 2022/8/16

Y1 - 2022/8/16

N2 - 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.

AB - 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.

UR - https://www.scopus.com/pages/publications/85137214091

U2 - 10.3389/fimmu.2022.973702

DO - 10.3389/fimmu.2022.973702

M3 - Scientific review articles

C2 - 36059476

AN - SCOPUS:85137214091

SN - 1664-3224

VL - 13

JO - Frontiers in Immunology

JF - Frontiers in Immunology

M1 - 973702

ER -

The relevance of complement in pemphigoid diseases: A critical appraisal

Abstract

Funding

UN SDGs

Research Areas and Centers

DFG Research Classification Scheme

Access to Document

Other files and links

Fingerprint

CRC 1526, PANTAU: Pathomechanisms of Antibody-mediated Autoimmunity

Cite this

The relevance of complement in pemphigoid diseases: A critical appraisal

Abstract

Funding

UN SDGs

Research Areas and Centers

DFG Research Classification Scheme

Access to Document

Other files and links

Fingerprint

Projects

CRC 1526, PANTAU: Pathomechanisms of Antibody-mediated Autoimmunity

Cite this