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SJS and TEN are nowadays considered variants of one disease entity with varying degrees of severity called epidermal necrolysis (EN). EN is associated with high morbidity and mortality and constitutes a major disease burden for affected patients. The guideline \u201cDiagnosis and treatment of epidermal necrolysis (Stevens\u2010Johnson syndrome and toxic epidermal necrolysis)\u201d was developed under systematic consideration of existing scientific literature and in a formal consensus process according to regulations issued by the Association of Scientific Medical Societies in Germany (AWMF) to establish an evidence\u2010based framework to support clinical decision\u2010making. The interdisciplinary guideline commission consisted of representatives from various specialist societies and patient representatives. The guideline is aimed at specialists in the fields of dermatology, ophthalmology, plastic surgery, intensive care, and pediatrics in hospitals and offices, as well as other medical speciallved in the diagnosis and treatment of EN. The guideline is also aimed at patients, their relatives, insurance funds, and policymakers. This first part focuses on the diagnostic aspects, the initial management as well as the immunomodulating systemic therapy.<\/jats:p>","DOI":"10.1111\/ddg.15515","type":"journal-article","created":{"date-parts":[[2024,9,24]],"date-time":"2024-09-24T02:36:09Z","timestamp":1727145369000},"page":"1448-1466","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens\u2010Johnson syndrome and toxic epidermal necrolysis) \u2013 Part 1: Diagnosis, initial management, and immunomodulating systemic therapy"],"prefix":"10.1111","volume":"22","author":[{"given":"Ruben","family":"Heuer","sequence":"first","affiliation":[{"name":"Department of Dermatology Venereology and Allergology Division of Evidence Based Medicine (dEBM) Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu 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