Pemphigus Vulgaris

Pemphigus vulgaris is a life-threatening bullous disease characterized by acantholysis resulting in the formation of intraepithelial blebs in the mucous membranes and skin. It is a chronic autoimmune bullous dermatosis caused by the production of autoantibodies against desmoglein 1 and 3. It often begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and drooping blisters that may spread to the skin. If there is clinical suspicion, the diagnosis can be confirmed by cytological examination, histopathological examination, direct and indirect immunofluorescence tests. Before the introduction of corticosteroids, PV was fatal due to dehydration or secondary systemic infections. The mainstay of treatment is still systemic steroids. Immunosuppressants such as azathioprine, mycophenolate mofetil and methotrexate, high-dose intravenous immunoglobulins, CD20 monoclonal antibody Rituximab treatments are used as an adjuvant with steroids in suitable patients and successful results are obtained