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

    September 2019 in “ Journal of Investigative Dermatology
    Roberta Lotti, Claudio Giacinto Atene, Alessandra Marconi, Marika Quadri, Elisabetta Palazzo, Giuliana Di Rocco, Tommaso Zanocco‐Marani, Carlo Pincelli
    TLDR Anti-Desmocollin 3 antibodies can cause pemphigus-like symptoms in mice.
    The study developed a mouse model to investigate the role of anti-Desmocollin 3 (DSC3) antibodies in pemphigus, an autoimmune blistering disease. By transferring DSC3 and/or Desmoglein 3 (DSG3) lymphocytes to immunodeficient mice, researchers found that anti-DSC3 antibodies alone could induce a pathological phenotype with erythema and patchy hair loss, resembling atypical pemphigus. The combination of anti-DSC3 and anti-DSG3 antibodies resulted in a more severe condition with frequent erosions, exacerbated erythema, and alopecia. Histological analysis revealed classical acantholysis, focal epidermal spongiosis, and dermal inflammatory cell infiltration. The DSC3/DSG3 model also showed a slower response to prednisolone treatment. This model supported the idea that antigens other than desmogleins could cause different pemphigus phenotypes in humans.
    Discuss this study in the Community →