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 symptoms like erythema and patchy hair loss, suggesting a link to atypical pemphigus. When both anti-DSC3 and anti-DSG3 antibodies were present, the mice exhibited more severe symptoms, including frequent erosions and exacerbated alopecia. Histological analysis showed classical acantholysis and inflammatory cell infiltration. The DSC3/DSG3 model also had a slower response to prednisolone compared to the DSC3 model. This research supported the idea that antigens other than desmogleins might cause different pemphigus phenotypes in humans.