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2018 ; 5
(ä): 201
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The Role of Eosinophils in Bullous Pemphigoid: A Developing Model of Eosinophil
Pathogenicity in Mucocutaneous Disease
#MMPMID30042946
Amber KT
; Valdebran M
; Kridin K
; Grando SA
Front Med (Lausanne)
2018[]; 5
(ä): 201
PMID30042946
show ga
Bullous pemphigoid (BP) is an autoimmune blistering disease which carries a
significant mortality and morbidity. While historically BP has been characterized
as an IgG driven disease mediated by anti-BP180 and BP230 IgG autoantibodies,
developments in recent years have further elucidated the role of eosinophils and
IgE autoantibodies. In fact, eosinophil infiltration and eosinophilic spongiosis
are prominent features in BP. Several observations support a pathogenic role of
eosinophils in BP: IL-5, eotaxin, and eosinophil-colony stimulating factor are
present in blister fluid; eosinophils line the dermo-epidermal junction (DEJ) in
the presence of BP serum, metalloprotease-9 is released by eosinophils at the
site of blisters; eosinophil degranulation proteins are found on the affected
basement membrane zone as well as in serum corresponding with clinical disease;
eosinophil extracellular DNA traps directed against the basement membrane zone
are present, IL-5 activated eosinophils cause separation of the DEJ in the
presence of BP serum; and eosinophils are the necessary cell required to drive
anti-BP180 IgE mediated skin blistering. Still, it is likely that eosinophils
contribute to the pathogenesis of BP in numerous other ways that have yet to be
explored based on the known biology of eosinophils. We herein will review the
role of eosinophils in BP and provide a framework for understanding eosinophil
pathogenic mechanisms in mucocutaneous disease.