Pemphigus is a severe, potentially fatal bullous skin disease, caused by desmoglein autoantibody production and immune-mediated regulation of T-cells subsets. Conventional therapy including systemic corticosteroids with or without other immunosupressants causes numerous adverse effects and becomes inefficient in refractory patients. In this work, the authors showed a modern view on the pathogenesis ofpemphigus. This article describes the detailed action mechanism of rituximab, a chimeric monoclonal antibody directed against CD20 antigen of B-cells. The authors conduct the results of meta-analyses of rituximab's efficiency in pemphigus patients. Moreover, in this article, the authors consider new promising treatment tions and potential targets for biological therapy of pemphigus diseases.