Pemphigus vulgaris is a rare, chronic vesiculobullous disease brought on by the development of autoantibodies against desmogleins 1 and 3. It is an immunoglobulin G-mediated autoimmune disorder of stratified squamous epithelia characterised by vesicles, bullae and erosions caused by acantholysis. Pemphigus vulgaris is the most prevalent and severe type of pemphigus, and it typically affects people between the ages of 40 and 60. Its onset and progression are determined by a complex interplay of predisposing and inducing factors. Hereditary susceptibility has a polygenic foundation, and, while necessary, is insufficient to start the autoimmune mechanism. Inducing external and internal factors such as medication intake, viral infections, physical agents, nutrition and stress appear to be crucial in initiating the disease. In most cases, pemphigus vulgaris begins with excruciatingly painful erosions of the oral mucosa. The flaccid cutaneous bullae that more than half of the patients experience eventually turn into oozing erosions on erythematous skin. Cutaneous erosions typically are covered with crusts and heal without leaving scars. A clinical variation known as pemphigus vegetans is characterised by the development of vegetating lesions in the deep skin creases. Histopathological analysis, as well as direct immunofluorescence studies and serological testing, can corroborate clinical suspicion. Systemic corticosteroids, frequently coupled with immunosuppressive adjuvants like mycophenolate mofetil, azathioprine and the anti-CD20 monoclonal antibody rituximab, are the current first-line therapy for pemphigus vulgaris. The illness typically follows a chronic-relapsing pattern, with possibly devastating consequences for the patients' quality of life.