A Comprehensive Review on Pemphigus Vulgaris

Pemphigus vulgaris is a life threatening chronic autoimmune disease characterized by the formation of intraepithelial blisters on the skin and mucous membranes. Pemphigus vulgaris initially manifests in the form of intraoral lesions which spread to other mucous membranes and the skin. Pemphigus vulgaris (PV), the most common and important variant, is an autoimmune blistering disease characterized by circulating pathogenic IgG (immunoglobin) antibodies against desmoglein 3 (Dsg3), about half the patients also having Dsg1 autoantibodies. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immunostaining examinations, is essential to the diagnosis. The diagnosis is based on clinical findings and laboratory analyses, and it is usually treated by the combined administration of corticosteroids and immunosuppressants. Detection of the oral lesions can result in an earlier diagnosis. The current review focuses on the etiopathogenesis, diagnosis and current treatment of pemphigus vulgaris.