Models of pemphigus vulgaris

Pemphigus vulgaris (PV) is a life-threatening blistering autoimmune disease of the skin and mucous membranes characterized by autoantibodies (autoAbs) against desmoglein 3 (Dsg3), a desmosomal adhesion protein expressed on the surface of epidermal keratinocytes. Loss of adhesion between epidermal keratinocytes leads to extensive blisters and painful erosions of mucous membranes and skin (Fig. 1). Recently developed in vitro and in vivo models of PV contributed significantly to a better understanding of its pathogenesis including the pathogenic role of autoAbs and the regulation of autoreactive B- and T-cell activation.

Section editor:Daniel Altmann – Human Disease Immunogenetics Group, Hammersmith Hospital, London, UK