Pseudo Pemphigus Phenotypes in Mice with Inactivated Desmoglein 3

The study by Kountikov et al. investigated a spontaneous mutation in the Dsg3 gene in mice, leading to hypomorphic expression of a truncated desmoglein 3 protein. The affected mice exhibited cyclic hair loss, obstructed airways, severe immunodeficiency, and deep tongue ulcers, but did not show the characteristic blistering of pemphigus vulgaris (PV). These findings challenged the hypothesis that Dsg3 is the principal desmosomal cadherin responsible for keratinocyte cohesion and suggested that other factors might contribute to the PV phenotype, highlighting the need for further research into the multipathogenic nature of PV.