Updates in Pathogenesis of Pemphigus Vulgaris

Autoantibodies are key pathogenic factors involved in the development of pemphigus vulgaris (PV). Recent advances in research have shed light on PV pathogenesis and may ultimately facilitate its tailored treatment. This review synthesizes evidence on the pathogenesis of PV, mainly from the past 5 years. The key mechanisms involved in PV development can be categorized into three major aspects. First, components that facilitate B cell differentiation and autoantibody production play a critical role. Second, the pathogenic effects of autoantibodies are mediated through direct damage or activation of signaling pathways, including Ca²⁺ influx, the JAK/STAT pathway, the p38MAPK pathway, the EGFR pathway, and apoptosis-related pathways. Notably, targeted treatments towards these mechanisms such as dupilumab also demonstrate promising efficacy. Third, additional contributing factors, including genetic factors, epigenetic factors, changes in protein expression, and microbial dysbiosis, further elucidate the complexity of PV pathogenesis.