043 A novel tool to analyse the pathogenic impact of IgG binding to extracellular domain 5 of Desmoglein 3

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Pemphigus vulgaris (PV) is a severe autoimmune blistering disease characterized by autoantibodies (auto-ab) against the desmosomal adhesion molecules desmoglein3 (Dsg3) and Dsg1. Binding of Dsg specific auto-ab to target structures induces an interruption of the desmosomal integrity which ultimately results in the clinical manifestation of flaccid blisters and erosions in PV patients. Underlying mechanisms inducing blister formation upon binding of Dsg-specific auto-ab are largely unknown. Numerous studies demonstrated the pathogenicity of auto-ab specific for the amino-terminal region (extracellular domain 1, EC1) of Dsg3.