Development of a Desmocollin-3 Active Mouse Model Recapitulating Human Atypical Pemphigus

The study developed a mouse model to investigate the role of anti-Desmocollin 3 (DSC3) antibodies in pemphigus, an autoimmune blistering disease. The model involved transferring DSC3 and/or Desmoglein 3 (DSG3) lymphocytes to immunodeficient mice. Results indicated that anti-DSC3 antibodies alone could induce a pemphigus-like phenotype with erythema and patchy hair loss, differing from the DSG3 model. The presence of both antibodies led to a more severe condition with frequent erosions and exacerbated symptoms. Histological analysis showed classical acantholysis, epidermal spongiosis, and dermal inflammation. The DSC3/DSG3 model also had a slower response to prednisolone treatment, suggesting that antigens other than desmogleins might contribute to different pemphigus phenotypes.