The relevance of complement in pemphigoid diseases: A critical appraisal
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 locate...
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| Format: | Article |
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Frontiers Media S.A.
2022-08-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2022.973702/full |
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| author | Cristian Papara Cristian Papara Christian M. Karsten Hideyuki Ujiie Enno Schmidt Enno Schmidt Leon F. Schmidt-Jiménez Adrian Baican Patricia C. Freire Kentaro Izumi Katja Bieber Matthias Peipp Admar Verschoor Admar Verschoor Ralf J. Ludwig Ralf J. Ludwig Jörg Köhl Jörg Köhl Detlef Zillikens Christoph M. Hammers Christoph M. Hammers |
| author_facet | Cristian Papara Cristian Papara Christian M. Karsten Hideyuki Ujiie Enno Schmidt Enno Schmidt Leon F. Schmidt-Jiménez Adrian Baican Patricia C. Freire Kentaro Izumi Katja Bieber Matthias Peipp Admar Verschoor Admar Verschoor Ralf J. Ludwig Ralf J. Ludwig Jörg Köhl Jörg Köhl Detlef Zillikens Christoph M. Hammers Christoph M. Hammers |
| author_sort | Cristian Papara |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-14T02:54:51Z |
| format | Article |
| id | doaj.art-8a3b5b23a04742fd9e16c0d69a776710 |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-04-14T02:54:51Z |
| publishDate | 2022-08-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-8a3b5b23a04742fd9e16c0d69a7767102022-12-22T02:16:08ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-08-011310.3389/fimmu.2022.973702973702The relevance of complement in pemphigoid diseases: A critical appraisalCristian Papara0Cristian Papara1Christian M. Karsten2Hideyuki Ujiie3Enno Schmidt4Enno Schmidt5Leon F. Schmidt-Jiménez6Adrian Baican7Patricia C. Freire8Kentaro Izumi9Katja Bieber10Matthias Peipp11Admar Verschoor12Admar Verschoor13Ralf J. Ludwig14Ralf J. Ludwig15Jörg Köhl16Jörg Köhl17Detlef Zillikens18Christoph M. Hammers19Christoph M. Hammers20Department of Dermatology, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, RomaniaInstitute of Systemic Inflammation Research, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, JapanDepartment of Dermatology, University of Lübeck, Lübeck, GermanyLübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, GermanyLübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, GermanyDepartment of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, RomaniaInstitute of Systemic Inflammation Research, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, JapanLübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, GermanyDivision of Antibody-Based Immunotherapy, Department of Medicine II, Christian-Albrechts-University of Kiel and University Medical Center Schleswig-Holstein, Kiel, GermanyDepartment of Dermatology, University of Lübeck, Lübeck, GermanyDepartment of Otorhinolaryngology, Klinikum rechts der Isar, Technical University Munich, Munich, GermanyDepartment of Dermatology, University of Lübeck, Lübeck, GermanyLübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, GermanyInstitute of Systemic Inflammation Research, University of Lübeck, Lübeck, GermanyDivision of Immunobiology, Cincinnati Children’s Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, OH, United StatesDepartment of Dermatology, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, University of Lübeck, Lübeck, GermanyLübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, GermanyPemphigoid 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.https://www.frontiersin.org/articles/10.3389/fimmu.2022.973702/fullcomplementrelevancepemphigoidbullous pemphigoidBPEBA |
| spellingShingle | Cristian Papara Cristian Papara Christian M. Karsten Hideyuki Ujiie Enno Schmidt Enno Schmidt Leon F. Schmidt-Jiménez Adrian Baican Patricia C. Freire Kentaro Izumi Katja Bieber Matthias Peipp Admar Verschoor Admar Verschoor Ralf J. Ludwig Ralf J. Ludwig Jörg Köhl Jörg Köhl Detlef Zillikens Christoph M. Hammers Christoph M. Hammers The relevance of complement in pemphigoid diseases: A critical appraisal Frontiers in Immunology complement relevance pemphigoid bullous pemphigoid BP EBA |
| title | The relevance of complement in pemphigoid diseases: A critical appraisal |
| title_full | The relevance of complement in pemphigoid diseases: A critical appraisal |
| title_fullStr | The relevance of complement in pemphigoid diseases: A critical appraisal |
| title_full_unstemmed | The relevance of complement in pemphigoid diseases: A critical appraisal |
| title_short | The relevance of complement in pemphigoid diseases: A critical appraisal |
| title_sort | relevance of complement in pemphigoid diseases a critical appraisal |
| topic | complement relevance pemphigoid bullous pemphigoid BP EBA |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2022.973702/full |
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