009 Human desmocollin 3-specific IgG antibodies are pathogenic in a humanized HLA-class II transgenic mouse model of pemphigus

Abstract

Pemphigus is a potentially lethal autoimmune bullous skin disorder, which is associated with IgG autoantibodies against desmoglein 3 (Dsg3) and Dsg1. Notably, a subset of pemphigus patients presents with a similar clinical phenotype in the absence of anti-Dsg IgG, suggesting the presence of serum IgG reactive with desmosomal components other than Dsg1 or Dsg3. We and others have previously shown that such patients have serum IgG autoantibodies against desmocollin 3 (Dsc3), a component of desmosomes, that induce loss of keratinocyte adhesion ex vivo.