Review of Pemphigus Vulgaris Management

Pemphigus Vulgaris (PV) is an autoimmune blistering disease that is characterized by painful erosions and flaccid blisters involving the mucous membranes and skin (Figure 1). The production of pathogenic immunoglobulin autoantibodies, mainly IgG4, against the desmosomal cadherins desmoglein 1 (Dsg-1) and desmoglein 3 (Dsg‑3), lead to the loss of epidermal keratinocyte adhesion. In the mucosal dominant type, Dsg-3 antibodies are present, while in the mucocutaneous type, antibodies against both desmoglein 1 and 3 are found. Diagnosing PV requires compatible clinical features, histopathological examination of the involved skin, and the detection of autoantibodies by indirect immunofluorescence of non-affected peri-lesional skin. The pemphigus disease area index (PDAI) and the autoimmune bullous skin disorder intensity score (ABSIS) provide a standardized assessment of disease extent and response to treatment. A treatment algorithm has been proposed for PV (Figure 2 and Figure 3). In addition, supportive management is also important (Table 1).