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2018 ; 9
(ä): 407
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The Autoimmune Skin Disease Bullous Pemphigoid: The Role of Mast Cells in
Autoantibody-Induced Tissue Injury
#MMPMID29545809
Fang H
; Zhang Y
; Li N
; Wang G
; Liu Z
Front Immunol
2018[]; 9
(ä): 407
PMID29545809
show ga
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. By 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 the 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.