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

Researchers developed a mouse model to study atypical pemphigus by focusing on the role of anti-Desmocollin 3 (DSC3) antibodies. This model involved transferring DSC3 and/or Desmoglein 3 (DSG3) lymphocytes to immunodeficient mice. The study found that anti-DSC3 antibodies alone could induce symptoms similar to pemphigus, such as erythema and patchy hair loss, but with distinct differences from the DSG3 model. When both anti-DSC3 and anti-DSG3 antibodies were present, the mice exhibited more severe symptoms, including frequent foreleg erosions and exacerbated erythema and alopecia. Histologically, the DSC3/DSG3 model showed classical acantholysis, focal epidermal spongiosis, and dermal inflammatory cell infiltration. The DSC3/DSG3 model also had a slower response to prednisolone treatment compared to the DSC3 model. This study concluded that antigens other than desmogleins might contribute to different pemphigus phenotypes in humans.