Refractory pemphigus vulgaris with extrapulmonary tuberculosis: A challenging case management with rituximab

Abstract

Pemphigus vulgaris is an autoimmune acantholytic blistering disease that affects the skin and mucous membranes and that is due to IgG autoantibodies targeted against adhesion molecules such as desmoglein 1 and 3 in the epidermis. It follows a chronic course with remissions and exacerbations affecting the patient’s quality of life. Since the advent of immunosuppressants, the prognosis of pemphigus has improved but mortality still persists. Long-term use of high-dose corticosteroids and immunosuppressants leads to serious adverse events. Recently, rituximab, a chimeric anti-CD20 monoclonal antibody, which causes B-cell depletion, has been shown to improve disease remission rates with faster tapering of steroids compared to the conventional treatment. Herein, we present a case of refractory pemphigus vulgaris with extrapulmonary tuberculosis and myelosuppression, focusing on unmet challenges due to high-dose systemic steroids, azathioprine, and successful management of the case with rituximab.