Major stages of the pathogenesis of true acantholytic pemphigus are interpreted in terms of autoimmune pathology; therefore, treatment of this group of diseases remains pathogenetic and is aimed at suppressing the synthesis of autoantibodies against keratinocyte proteins. Treatment with the use of systemic glucocorticosteroids is currently the main method of treatment for true acantholytic pemphigus. To reduce the course dose of glucocorticosteroids, decrease the risk of adverse effect development and achieve long-term remission, further studies of disease pathogenesis and development of new treatment methods for reducing the doses of glucocorticosteroids are of great importance.