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.
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