In Vitro Pathogenicity Assay for Anti-desmoglein Autoantibodies in Pemphigus

Patients with pemphigus have circulating anti-desmoglein (Dsg) 1 and/or Dsg3 autoantibodies that induce blister formation on the skin and mucous membrane. We describe here two assays that measure the pathogenic strength of autoantibodies in blister formation: an in vitro dissociation assay using primary human epidermal keratinocytes to assess pathogenicity of anti-Dsg3 autoantibodies, and an alternative method whereby anti-Dsg3 and Dsg1 autoantibodies are injected into organ-cultured human skin specimen.