Pemphigus Vulgaris

Pemphigus vulgaris (PV) is the main intraepithelial blistering autoimmune disease of the skin and surface-close mucosae characterized by pathogenic autoantibodies interfering with the desmosomal cell-cell adhesion molecules desmoglein 1 and 3. The oral cavity is the main affected area and the initial site to be involved in most patients with PV. Clinical symptoms include enanthema, blisters, and partly painful erosions and ulcers localized anywhere on the oral mucosa, which may or may not be accompanied or followed by cutaneous blistering. Diagnosis is based on clinical manifestations and confirmed by histological and immunological testing including direct immunofluorescence microscopy and desmoglein 1/3-specific enzyme-linked immunosorbent assay. The current first-line treatment is systemic corticosteroids and the anti-CD20 antibody rituximab, while other therapies comprise corticosteroid-sparing adjuvants such as azathioprine, mycophenolate mofetil, high-dose intravenous immunoglobulins, and immunoadsorption.