Bullous Pemphigoid: A Prototypical Antibody-Mediated Organ-Specific Autoimmune Disease

Abstract

Bullous pemphigoid (BP) is a prototypical organ-specific autoimmune disease. Autoantibodies unfold their blister-inducing potential by triggering an Fcgamma-dependent inflammatory reaction. The study by Iwata et al. in this issue provides the first direct evidence that IgG autoantibodies from BP patients may also weaken cell-matrix adhesion by depleting BP180/type XVII collagen from cultured keratinocytes. These novel findings shed new light on additional mechanisms of blister formation in pemphigoid diseases and open the way for further informative studies.