Pemphigus Vulgaris

Pemphigus Vulgaris is an autoimmune blistering disease characterized by production of IgG autoantibodies against desmogleins, adhesion molecules essential for cell-to-cell adhesion. Antibodies target primarily desmoglein 3 (mucosal involvement) and sometimes also desmoglein 1 (mucocutaneous disease). Antibody binding disrupts cell adhesion, leading to acantholysis (loss of cell-to-cell adhesion) and intraepidermal blister formation. Clinically, patients typically present with painful oral erosions and ulcerations, often the initial manifestation. Flaccid blisters then develop on skin, characteristically rupturing easily to leave painful, slow-healing erosions. The condition can become severe with extensive erosions affecting eating, breathing, and ability to care for self-care. Erosions are susceptible to secondary bacterial infection, leading to sepsis risk. Suprabasal acantholysis (within lower epidermis) is characteristic histopathology. In mucosal-dominant disease, oral involvement predominates. In mucocutaneous disease, both oral and skin involvement occurs. Untreated disease was historically fatal, with mortality from secondary infection or malnutrition.