Therapeutic effects of Fc gamma RIV inhibition are mediated by selectively blocking immune complex-induced neutrophil activation in epidermolysis bullosa acquisita.

ABSTRACT: Epidermolysis bullosa acquisita (EBA) is a subepidermal autoimmune bullous disease caused by autoantibodies targeting type VII collagen (COL7). It is characterized by inflammation and subepidermal blistering mainly through immune complex (IC)-mediated activation of neutrophils. In experimental EBA, binding of neutrophils to ICs in the skin and induction of clinical disease depends on the expression of the Fc gamma receptor (FcγR) IV. As activating FcγR mediate both neutrophil extravasation and activation, we used multiphoton imaging to obtain further insights into the mechanistic contribution of FcγRIV in the pathogenesis of EBA. First, we demonstrated that blocking FcγRIV function completely protects LysM-eGFP mice against induction of antibody transfer-induced EBA. To visualize the inter

SUBMITTER: Haeger SC 

PROVIDER: S-EPMC9606225 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature