Pemphigus vulgaris (PV) is a rare, chronic autoimmune blistering disorder affecting the skin and mucous membranes. It is characterized by the production of IgG autoantibodies against desmoglein-1 and desmoglein-3, which are essential components of desmosomes responsible for cell-to-cell adhesion in the epidermis. Loss of adhesion between keratinocytes leads to acantholysis and the formation of painful blisters and erosions, commonly beginning in the oral cavity before involving the skin. PV most frequently affects middle-aged individuals and shows higher prevalence among certain ethnic groups, including those of Mediterranean and Ashkenazi Jewish descent. Diagnosis is confirmed by clinical features, histopathology, and direct immunofluorescence studies. Early diagnosis and prompt treatment with systemic corticosteroids and immunosuppressive agents, such as azathioprine or rituximab, significantly reduce morbidity and mortality. Although once associated with high fatality rates, advances in immunotherapy have markedly improved patient prognosis.