S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis) - Part 1: Diagnosis, initial management, and immunomodulating systemic therapy

Ruben Heuer¹, Maren Paulmann², Thorsten Annecke³, Björn Behr^{4

5}, Katharina Boch⁶, Anja M Boos⁷, Knut Brockow⁸, Lars E French⁹, Jochen Gille¹⁰, Verena Gundlach¹¹, Bernd Hartmann¹², Peter Höger¹³, Silke C Hofmann¹⁴, Tobias Klein¹⁵, Marcus Lehnhardt⁴, Yvonne Liß¹⁶, Philip Maier¹⁷, Philipp Mandel¹⁸, Nicos Marathovouniotis¹⁵, Finnja Marlok¹⁹, Hans Mittelviefhaus⁹, Uwe Pleyer^{20

21}, Marie Pradeau¹, Katharina Rall²², Siegbert Rieg²³, Heike Rittner²⁴, Frank Sander¹², Stefan Schnitzler²⁵, Christina Schut²⁶, Annette Stolle²⁷, Artem Vorobyev⁶, Bettina Wedi²⁸, Johannes Weiss²⁹, Maximilian Zepp³⁰, Mirjana Ziemer³¹, Maja Mockenhaupt², Alexander Nast¹

Affiliations

¹ Department of Dermatology, Venereology and Allergology, Division of Evidence Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
² Department of Dermatology and Venereology, Dokumentationszentrum schwerer Hautreaktionen (dZh), University Medical Center Freiburg, Freiburg, Germany.
³ Department of Anesthesiology and Critical Care Medicine, Merheim Medical Center, Hospital of Witten/Herdecke University, Cologne, Germany.
⁴ Department of Plastic and Hand Surgery, Burn Center, Sarcoma Center, BG University Hospital Bergmannsheil Ruhr University Bochum, Bochum, Germany.
⁵ Department of Plastic, Reconstructive & Aesthetic Surgery, KEM Evangelische Kliniken Essen-Mitte, Evangelische Huyssens-Stiftung Essen-Huttrop, Essen, Germany.
⁶ Department of Dermatology, Allergology and Venereology, University Hospital Schleswig-Holstein, Campus Lübeck, Germany.
⁷ Department of Plastic Surgery, Hand Surgery - Burn Center, University Hospital RWTH Aachen, Aachen, Germany.
⁸ Department of Dermatology and Allergology, University Medical Center, Technical University of Munich, Munich, Germany.
⁹ Department of Dermatology and Allergy, Ludwig Maximilian University Munich, Munich, Germany.
¹⁰ Department of Anesthesiology, Intensive Care Medicine and Pain Therapy, St. Georg Hospital Leipzig, Leipzig, Germany.
¹¹ Heart Center Niederrhein - Department of Cardiac Surgery and Cardiovascular Surgery, Helios Hospital Krefeld, Krefeld, Germany.
¹² Department of Plastic Surgery, Burn Centre, BG Klinikum Unfallkrankenhaus Berlin, Berlin, Germany.
¹³ Department of Pediatric Dermatology/Allergology, Catholic Children's Hospital Wilhelmstift Hamburg, Hamburg, Germany.
¹⁴ Center for Dermatology, Allergology and Dermatosurgery, Helios University Hospital Wuppertal, University of Witten/Herdecke, Wuppertal, Germany.
¹⁵ Department of Pediatric Surgery and Pediatric Urology, Children's Hospital of Cologne, Cologne, Germany.
¹⁶ Office Dr. Jana Filser, Mannheim, Germany.
¹⁷ Eye Center, University Medical Center Freiburg, Freiburg, Germany.
¹⁸ Department of Urology, University Hospital Frankfurt, Frankfurt am Main, Germany.
¹⁹ Patient Representative, Untermünkheim, Germany.
²⁰ Department of Ophthalmology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
²¹ Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.
²² Department of Women's Health, University Hospital Tübingen, Tübingen, Germany.
²³ Division of Infectious Diseases, Department of Medicine II -, Gastroenterology, Hepatology, Endocrinology and Infectiology, University Medical Center Freiburg, Freiburg, Germany.
²⁴ Department of Anesthesiology, Intensive Care, Emergency and Pain Medicine, Center for Interdisciplinary Pain Medicine, University Hospital Würzburg, Würzburg, Germany.
²⁵ Department of Surgical Intensive Care and Intermediate Care, University Hospital RWTH Aachen, Aachen, Germany.
²⁶ Institute of Medical Psychology, Justus Liebig University Gießen, Gießen, Germany.
²⁷ Andreas Wentzensen Research Institute, BG Hospital Ludwigshafen, Ludwigshafen, Germany.
²⁸ Department of Dermatology, Allergology and Venereology, Hannover Medical School, Hannover, Germany.
²⁹ Department of Dermatology and Allergology, University Hospital Ulm, Ulm, Germany.
³⁰ Patient Representative, Kaiserslautern, Germany.
³¹ Department of Dermatology, Venereology and Allergology, University Hospital Leipzig, Leipzig, Germany.

PMID: 39314017
DOI: 10.1111/ddg.15515

Practice Guideline

S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis) - Part 1: Diagnosis, initial management, and immunomodulating systemic therapy

Ruben Heuer et al. J Dtsch Dermatol Ges. 2024 Oct.

. 2024 Oct;22(10):1448-1466.

doi: 10.1111/ddg.15515. Epub 2024 Sep 23.

Authors

Affiliations

¹ Department of Dermatology, Venereology and Allergology, Division of Evidence Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
² Department of Dermatology and Venereology, Dokumentationszentrum schwerer Hautreaktionen (dZh), University Medical Center Freiburg, Freiburg, Germany.
³ Department of Anesthesiology and Critical Care Medicine, Merheim Medical Center, Hospital of Witten/Herdecke University, Cologne, Germany.
⁴ Department of Plastic and Hand Surgery, Burn Center, Sarcoma Center, BG University Hospital Bergmannsheil Ruhr University Bochum, Bochum, Germany.
⁵ Department of Plastic, Reconstructive & Aesthetic Surgery, KEM Evangelische Kliniken Essen-Mitte, Evangelische Huyssens-Stiftung Essen-Huttrop, Essen, Germany.
⁶ Department of Dermatology, Allergology and Venereology, University Hospital Schleswig-Holstein, Campus Lübeck, Germany.
⁷ Department of Plastic Surgery, Hand Surgery - Burn Center, University Hospital RWTH Aachen, Aachen, Germany.
⁸ Department of Dermatology and Allergology, University Medical Center, Technical University of Munich, Munich, Germany.
⁹ Department of Dermatology and Allergy, Ludwig Maximilian University Munich, Munich, Germany.
¹⁰ Department of Anesthesiology, Intensive Care Medicine and Pain Therapy, St. Georg Hospital Leipzig, Leipzig, Germany.
¹¹ Heart Center Niederrhein - Department of Cardiac Surgery and Cardiovascular Surgery, Helios Hospital Krefeld, Krefeld, Germany.
¹² Department of Plastic Surgery, Burn Centre, BG Klinikum Unfallkrankenhaus Berlin, Berlin, Germany.
¹³ Department of Pediatric Dermatology/Allergology, Catholic Children's Hospital Wilhelmstift Hamburg, Hamburg, Germany.
¹⁴ Center for Dermatology, Allergology and Dermatosurgery, Helios University Hospital Wuppertal, University of Witten/Herdecke, Wuppertal, Germany.
¹⁵ Department of Pediatric Surgery and Pediatric Urology, Children's Hospital of Cologne, Cologne, Germany.
¹⁶ Office Dr. Jana Filser, Mannheim, Germany.
¹⁷ Eye Center, University Medical Center Freiburg, Freiburg, Germany.
¹⁸ Department of Urology, University Hospital Frankfurt, Frankfurt am Main, Germany.
¹⁹ Patient Representative, Untermünkheim, Germany.
²⁰ Department of Ophthalmology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
²¹ Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.
²² Department of Women's Health, University Hospital Tübingen, Tübingen, Germany.
²³ Division of Infectious Diseases, Department of Medicine II -, Gastroenterology, Hepatology, Endocrinology and Infectiology, University Medical Center Freiburg, Freiburg, Germany.
²⁴ Department of Anesthesiology, Intensive Care, Emergency and Pain Medicine, Center for Interdisciplinary Pain Medicine, University Hospital Würzburg, Würzburg, Germany.
²⁵ Department of Surgical Intensive Care and Intermediate Care, University Hospital RWTH Aachen, Aachen, Germany.
²⁶ Institute of Medical Psychology, Justus Liebig University Gießen, Gießen, Germany.
²⁷ Andreas Wentzensen Research Institute, BG Hospital Ludwigshafen, Ludwigshafen, Germany.
²⁸ Department of Dermatology, Allergology and Venereology, Hannover Medical School, Hannover, Germany.
²⁹ Department of Dermatology and Allergology, University Hospital Ulm, Ulm, Germany.
³⁰ Patient Representative, Kaiserslautern, Germany.
³¹ Department of Dermatology, Venereology and Allergology, University Hospital Leipzig, Leipzig, Germany.

PMID: 39314017
DOI: 10.1111/ddg.15515

Abstract

Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are rare, predominantly drug-induced, acute, life-threatening diseases of skin and mucosae. 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 "Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis)" 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-based framework to support clinical decision-making. 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.

Keywords: Drug reaction; Lyell syndrome; Stevens‐Johnson syndrome; epidermal necrolysis; toxic epidermal necrolysis.

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References

REFERENCES

1. Kaminski‐Hartenthaler A, Meerpohl JJ, Gartlehner G, et al. [GRADE guidelines: 14. Going from evidence to recommendations: the significance and presentation of recommendations]. Z Evid Fortbild Qual Gesundhwes. 2014;108:413‐420.
1. Lyell A. Toxic epidermal necrolysis (the scalded skin syndrome): a reappraisal. Br J Dermatol. 1979;100:69‐86.
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1. Bastuji‐Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens‐Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993;129:92‐96.
1. Auquier‐Dunant A, Mockenhaupt M, Naldi L, et al. Correlations between clinical patterns and causes of erythema multiforme majus, Stevens‐Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study. Arch Dermatol. 2002;138:1019‐1024.

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S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis) - Part 1: Diagnosis, initial management, and immunomodulating systemic therapy

Affiliations

S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis) - Part 1: Diagnosis, initial management, and immunomodulating systemic therapy

Authors

Affiliations

Abstract

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REFERENCES

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LinkOut - more resources

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Research Materials