Guidelines for the Management of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis 2009, Established by the Japanese Research Committee on Severe Adverse Reaction (J-SCAR) Supported by the Ministry of Health, Labour and Welfare of Japan

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe, sometimes life threatening disorders that seem to be variants of the same disease with different severities. Although the pathogenic mechanisms of these diseases remain unknown, the principal problem seems immunologic. Recently, the available published evidence indicates that immunomodulatory intervention with high-dose systemic corticosteroids, including pulse therapy, is effective in the acute phase of SJS and TEN. In spite of the severity of these diseases, there had not been widely accepted guidelines for management of these diseases. In the current guidelines, a prompt use of high-dose steroids in the early stage is emphasized. Infection control is critical to survival for the patients. When steroids are not effective enough, intravenous immunoglobulin, and/or plasma exchange, in addition to an increase of steroid dose, is recommended.