Epidermolysis bullosa acquisita: From pathophysiology to novel therapeutic options

Michael Kasperkiewicz; Christian D. Sadik; Katja Bieber; Saleh M. Ibrahim; Rudolf A. Manz; Enno Schmidt; Detlef Zillikens; Ralf J. Ludwig

doi:10.1038/JID.2015.356

Epidermolysis bullosa acquisita: From pathophysiology to novel therapeutic options

Michael Kasperkiewicz^*, Christian D. Sadik, Katja Bieber, Saleh M. Ibrahim, Rudolf A. Manz, Enno Schmidt, Detlef Zillikens, Ralf J. Ludwig

^*Corresponding author for this work

99 Citations (Scopus)

Abstract

Epidermolysis bullosa acquisita (EBA) is a prototypic organ-specific autoimmune disease induced by autoantibodies to type VII collagen causing muco-cutaneous blisters. In the inflammatory (bullous pemphigoid-like) EBA variant, autoantibody binding is followed by a lesional inflammatory cell infiltration, and the overall clinical picture may be indistinguishable from that of bullous pemphigoid, the latter being the most common autoimmune bullous disease. The last decade witnessed the development of several mouse models of inflammatory EBA that facilitated the elucidation of the pathogenesis of autoantibody-induced, cell-mediated subepidermal blistering diseases and identified new therapeutic targets for these and possibly other autoantibody-driven disorders.

Original language	English
Journal	Journal of Investigative Dermatology
Volume	136
Issue number	1
Pages (from-to)	24-33
Number of pages	10
ISSN	0022-202X
DOIs	https://doi.org/10.1038/JID.2015.356
Publication status	Published - 01.01.2016

Funding

We thank all colleagues who contributed to the studies reviewed in this article. Our own summarized work was supported by Deutsche Forschungsgemeinschaft (EXC 306/1 and 2; GRK 1727/1; GRK 1743/1; ZI 439/6-1 and 6-2; LU 877/5-1, 8-1, 9-1, 10-1; KA 3438/1-1; and SA 1960/3-1), University of Lübeck (Focus Program Autoimmunity and Research Training Grants E16-2012, E06-2013, and E22-2013), Doktor Robert Pfleger and Else Kröner-Fresenius Foundation, EFRE 122-09-017, and project research grants from Biotest, Dompé, Euroimmun, and Novartis.

UN SDGs

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

SDG 3 Good Health and Well-being

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10.1038/JID.2015.356

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title = "Epidermolysis bullosa acquisita: From pathophysiology to novel therapeutic options",

abstract = "Epidermolysis bullosa acquisita (EBA) is a prototypic organ-specific autoimmune disease induced by autoantibodies to type VII collagen causing muco-cutaneous blisters. In the inflammatory (bullous pemphigoid-like) EBA variant, autoantibody binding is followed by a lesional inflammatory cell infiltration, and the overall clinical picture may be indistinguishable from that of bullous pemphigoid, the latter being the most common autoimmune bullous disease. The last decade witnessed the development of several mouse models of inflammatory EBA that facilitated the elucidation of the pathogenesis of autoantibody-induced, cell-mediated subepidermal blistering diseases and identified new therapeutic targets for these and possibly other autoantibody-driven disorders.",

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AU - Zillikens, Detlef

AU - Ludwig, Ralf J.

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Epidermolysis bullosa acquisita: From pathophysiology to novel therapeutic options

Abstract

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UN SDGs

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