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Front. Immunol. | doi: 10.3389/fimmu.2018.00407

The autoimmune skin disease bullous pemphigoid: the role of mast cells in autoantibody-induced tissue injury

Hui Fang1, Yang Zhang2, Ning Li3,  Gang Wang1 and  Zhi Liu3*
  • 1Fourth Military Medical University, China
  • 2Second Affiliated Hospital of Xi'an Jiaotong University, China
  • 3University of North Carolina at Chapel Hill, United States

Bullous pemphigoid (BP) is an autoimmune and inflammatory skin disease associated with subepidermal blistering and autoantibodies directed against the hemidesmosomal components BP180 and BP230. Animal models of BP were developed by passively transferring anti-BP180 IgG into mice, which recapitulates the key features of human BP. Using these in vivo model systems, key cellular and molecular events leading to the BP disease phenotype are identified, including binding of pathogenic IgG to its target, complement activation of the classical pathway, mast cell degranulation, and infiltration and activation of neutrophils. Proteinases released by infiltrating neutrophils cleave BP180 and other hemidesmosome-associated proteins, causing DEJ separation. Mast cells and mast cell-derived mediators including inflammatory cytokines and proteases are increased in lesional skin and blister fluids of BP. BP animal model evidence also implicates mast cells in the pathogenesis of BP. However, recent studies questioned the pathogenic role of mast cells in autoimmune diseases such as multiple sclerosis, rheumatoid arthritis, and epidermolysis bullosa acquisita. This review highlights current knowledge on BP pathophysiology with a focus on a potential role for mast cells in BP, and mast cell-related critical issues needing to be addressed in the future.

Keywords: Autoantibodies, Bullous pemphigoid, hemidesmosome, Mast Cells, skin autoimmunity

Received: 29 Nov 2017; Accepted: 14 Feb 2018.

Edited by:

Falk Nimmerjahn, University of Erlangen-Nuremberg, Germany

Reviewed by:

Kempuraj Duraisamy, University of Missouri, United States
Frank Petersen, Forschungszentrum Borstel (LG), Germany  

Copyright: © 2018 Fang, Zhang, Li, Wang and Liu. 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 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.

* Correspondence: Prof. Zhi Liu, University of North Carolina at Chapel Hill, Chapel Hill, United States,