Pemphigus vulgaris (PV) encompasses a group of life-threatening autoimmune bullous diseases characterized by flaccid blisters and erosions of the mucous membrane and skin. The average age of onset is between 50 and 70 years. PV rarely occurs in children. The severity of the disease is based on its progressive course, which is accompanied by increased body catabolism with loss of body fluids and proteins and secondary bacterial infections that may lead to sepsis and cardiac failure. The mainstay of treatment remains corticosteroids with or without adjuvant therapy. Adjuvant therapy includes steroid-sparing agents and immunotherapy procedures. Due to the variation in severity, varied response to the conventional treatment protocols, and severe side effects, dermatologists all over the world have ventured into using new modalities of adjuvant therapies, such as intravenous immunoglobulins and therapeutic plasma exchange, to grapple the increasing number of patients with severe PV who show little or no response to conventional corticosteroid treatment. We report a case of severe PV in a patient who did not respond to prednisone and azathioprine therapy but was successfully treated with plasmapheresis.
J Med Case Reports. 2020;14(109) © 2020 BioMed Central, Ltd.