Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent a severe spectrum of rare mucocutaneous reactions that are primarily drug-induced and characterized by significant morbidity and mortality. These conditions manifest in extensive skin detachment, distinguishing them from other generalized skin eruptions. The rarity and severity of SJS/TEN underscore the importance of accurate diagnostic criteria and effective treatments, which are currently lacking consensus. This review proposes new diagnostic criteria to improve specificity and global applicability. Recent advances in understanding the immunopathogenesis of SJS/TEN are explored, emphasizing the role of drug-specific T-cell responses and human leucocyte antigen polymorphisms in disease onset. The review also addresses current therapeutic approaches, including controversies surrounding the use of immunosuppressive agents and the emerging role of tumour necrosis factor-α inhibitors. Novel therapeutic strategies targeting specific pathogenic mechanisms, such as necroptosis and specific immune cell pathways, are discussed. Furthermore, the development of new drugs based on these insights, including targeted monoclonal antibodies and inhibitors, are examined. The review concludes by advocating for more robust and coordinated efforts across multidisciplinary medical fields to develop effective treatments and diagnostic tools for SJS/TEN, with the aim of improving patient outcomes and understanding the disease and its mechanisms.
Stevens–Johnson syndrome (‘SJS’ for short) and toxic epidermal necrolysis (‘TEN’ for short) are rare but serious skin conditions. They are usually caused by a reaction to medication. They cause large areas of the skin to peel off, making them different to other conditions that affect the skin. It is important to have clear ways to diagnose and treat these different conditions. However, healthcare professionals do not all agree on the best methods to use. This review was done by researchers in the USA and the UK. It suggests new ways to diagnose SJS and TEN to make it easier and more accurate for doctors around the world. We looked at the latest research on what causes these conditions. We focused on how the body’s immune system reacts to certain drugs and genetic factors that might make some people more likely to get SJS or TEN. We looked at current treatments, including drugs that suppress the immune system. We then investigated new treatments that block specific parts of the immune response. We call for better cooperation among different medical specialties to develop more effective treatments and tools to diagnose SJS and TEN. This will help improve the lives of people affected by these severe conditions and increase our understanding of SJS and TEN.
© The Author(s) 2024. Published by Oxford University Press on behalf of British Association of Dermatologists.