Pemphigus vulgaris (PV) is an uncommon intraepidermal blistering disorder. The mean age of onset is between 40 and 60 years, and the incidence is 2.83 per million population worldwide. The oral mucosa is first affected later developed to skin involvement in the course of illness. It is commonly known that immunological and genetic factors affect the onset of PV. However, the illness may be brought on by or affected by external variables (drugs, food, viruses, etc.). The diagnosis requires a biopsy of the perilesional tissue along with histological and immunostaining analyses. Nikolsky’s sign is an important clinical diagnostic criteria. First-line treatment involves systemic corticosteroids, often at 1?mg/kg/day, combined with steroid-sparing agents such as azathioprine or mycophenolate mofetil. In refractory or severe cases, rituximab, IVIG, plasmapheresis, or immunoadsorption are effective adjuncts. With these advances, mortality has dropped from over 60–90% to approximately 5–15%. Continuing research into B cell–targeted biologics and optimized immunomodulatory regimens aims to enhance long term remission and reduce treatment related morbidity.
Pemphigus, Desmoglein, Immunoglobulins, plasmapheresis.
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