SJS, SJS/TEN and TEN are severe and uncommon adverse drug reactions, representing different manifestations within a spectrum of the same disease characterized by denudation of the skin and mucous membranes. The literature reports outcomes with systemic corticosteroids, intravenous immunoglobulin, cyclosporine, TNF-alpha inhibitors, plasmapheresis, and Janus kinase inhibitors.
Aim: To provide an updated overview of the comprehensive management of SJS/TEN, aiming to increase survival, reduce sequelae, and improve quality of life.
Methods: A systematic search was conducted between September 2024 and April 2025 in indexed databases, selecting 55 articles and 3 relevant clinical guidelines.
Results: The diagnosis of SJS/TEN should be considered in the presence of fever, lymphopenia, atypical cutaneous lesions, and a positive Nikolsky sign. Immediate discontinuation of suspected drugs and prompt skin biopsy are essential. It is recommended to use mortality prediction scores such as SCORTEN, ABCD-10 and CRISTEN. Patients should be hospitalized in an intensive care unit or burn center under strict life support and isolation protocols. Management must be multidisciplinary and includes supportive measures such as fluid replacement, temperature control, nutritional support, analgesia, thromboprophylaxis, and infection prevention. Systemic therapy is always recommended and, depending on response and disease progression, may include glucorticosteroids, intravenous immunoglobulin, cyclosporine, TNF-alpha inhibitors, plasmapheresis, or, in exceptional cases, JAK inhibitors. Follow-up during the first year is suggested to monitor for potential cutaneous, mucosal, visual, and psychological sequelae.
Conclusions: This study provides practical and updated recommendations for early diagnosis, prioritization of life support measures, and selection of therapeutic alternatives, with the goal of improving patient survival and quality of life. Early initiation of systemic therapy is crucial to improve patient prognosis.
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