Supportive care in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: an international, multidisciplinary Delphi-based consensus

M-C Brüggen; S T Le; S Walsh; A Toussi; N de Prost; A Ranki; B Didona; A Colin; B Horváth; E Brezinova; B Milpied; C Moss; C Bodemer; D Meyersburg; C Salavastru; G-S Tiplica; E Howard; E Bequignon; J N Bouwes Bavinck; J Newman; J Gueudry; M Nägeli; K Zaghbib; K Pallesen; A Bygum; P Joly; P Wolkenstein; S-L Chua; R Le Floch; N H Shear; C-Y Chu; N Hama; R Abe; W-H Chung; T Shiohara; M Ardern-Jones; P Romanelli; E J Phillips; R S Stern; J Cotliar; R G Micheletti; A Brassard; J T Schulz; R P Dodiuk-Gad; A R Dominguez; A S Paller; L Seminario-Vidal; A Mostaghimi; M H Noe; S Worswick; D Tartar; R Sheridan; B H Kaffenberger; K Shinkai; E Maverakis; L E French; S Ingen-Housz-Oro

doi:10.1111/bjd.19893

Supportive care in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: an international, multidisciplinary Delphi-based consensus

Br J Dermatol. 2021 Sep;185(3):616-626. doi: 10.1111/bjd.19893. Epub 2021 Apr 27.

Authors

M-C Brüggen^{1

2

3

4}, S T Le⁵, S Walsh^{4

6}, A Toussi⁵, N de Prost^{7

8}, A Ranki^{4

9}, B Didona^{4

10}, A Colin^{4

8

11}, B Horváth^{4

12}, E Brezinova^{4

13}, B Milpied^{4

8

14}, C Moss^{4

15}, C Bodemer^{4

8

16}, D Meyersburg^{4

17}, C Salavastru^{4

18}, G-S Tiplica^{4

19}, E Howard^{4

15}, E Bequignon^{4

20}, J N Bouwes Bavinck²¹, J Newman²², J Gueudry^{8

23}, M Nägeli¹, K Zaghbib^{8

24}, K Pallesen^{4

25}, A Bygum^{4

26}, P Joly^{4

8

27}, P Wolkenstein^{4

8

11}, S-L Chua^{4

28}, R Le Floch^{8

29}, N H Shear^{30

31}, C-Y Chu³², N Hama³³, R Abe³³, W-H Chung³⁴, T Shiohara³⁵, M Ardern-Jones³⁶, P Romanelli³⁷, E J Phillips³⁸, R S Stern³⁹, J Cotliar⁴⁰, R G Micheletti⁴¹, A Brassard⁵, J T Schulz⁴², R P Dodiuk-Gad³¹, A R Dominguez⁴³, A S Paller⁴⁴, L Seminario-Vidal⁴⁵, A Mostaghimi⁴⁶, M H Noe⁴⁶, S Worswick⁴⁷, D Tartar⁵, R Sheridan⁴⁸, B H Kaffenberger⁴⁹, K Shinkai⁵⁰, E Maverakis⁵, L E French^{4

36

51}, S Ingen-Housz-Oro^{4

8

11

52}

Affiliations

¹ Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
² Faculty of Medicine, University of Zurich, Zurich, Switzerland.
³ Christine Kühne Center for Allergy Research and Education, Davos, Switzerland.
⁴ ToxiTEN Group, European Reference Network for Rare Skin Diseases (ERN-skin), Paris, France.
⁵ Department of Dermatology, University of California, Davis, Sacramento, CA, USA.
⁶ Department of Dermatology, King's College Hospital, London, UK.
⁷ Intensive Care Unit, AP-HP, Henri Mondor Hospital, Créteil, France.
⁸ Toxic Bullous Dermatoses TOXIBUL Reference Centre, Filière FIMARAD, AP-HP, Henri Mondor Hospital, Créteil, France.
⁹ Department of Skin and Allergic Diseases, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland.
¹⁰ First Dermatology Division, Institute Dermopatico dell'Immacolata (I.D.I.) - IRCCS, Via Monti di Creta 104, Rome, 00167, Italy.
¹¹ Dermatology Department, AP-HP, Henri Mondor Hospital, Créteil, France.
¹² Department of Dermatology, Center for Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
¹³ Department of Dermatovenereology, St Ann's University Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
¹⁴ Department of Dermatology, CHU Bordeaux, Bordeaux, France.
¹⁵ Birmingham Children's Hospital and University of Birmingham, Birmingham, UK.
¹⁶ Department of Dermatology, AP-HP, Necker Hospital, Paris, France.
¹⁷ Department of Dermatology and Allergology, University Hospital Salzburg of the Paracelsus Medical University Salzburg, Salzburg, Austria.
¹⁸ Department of Paediatric Dermatology, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
¹⁹ Department of Dermatology II, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
²⁰ Department of Otorhinolaryngology and Head and Neck Surgery, AP-HP, Henri Mondor Hospital, Créteil, France.
²¹ Department of Dermatology, Leiden University Medical Center, Leiden, the Netherlands.
²² Macmillan Skin Cancer CNS, Normanby Building, Denmark Hill, London, UK.
²³ Ophthalmology Department, Hospital Charles Nicolle, EA7510, UFR Santé, Rouen University, Rouen, France.
²⁴ Department of Psychiatry, AP-HP, Henri Mondor-Albert Chenevier Hospitals, Cr, France.
²⁵ Department of Dermatology, Aarhus University Hospital, Palle Juul-Jensens, Aarhus, Denmark.
²⁶ Clinical Institute, University of Southern Denmark, Department of Clinical Genetics, Odense University Hospital, Odense, Denmark.
²⁷ Department of Dermatology, CHU Charles, Nicolle, Rouen, France.
²⁸ Queen Elizabeth Hospital Birmingham, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK.
²⁹ Réanimation Chirurgicale et des Brûlés, PTMC, CHU Nantes, Nantes, France.
³⁰ Division of Clinical Pharmacology and Toxicology, University of Toronto, Toronto, ON, Canada.
³¹ Division of Dermatology, Sunnybrook Health Sciences Centre, Toronto, ON, Canada.
³² Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
³³ Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
³⁴ Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan.
³⁵ Department of Dermatology, Kyorin University School of Medicine, Tokyo, Japan.
³⁶ Clinical Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, UK.
³⁷ Department of Dermatology and Cutaneous Surgery, Miller School of Medicine, University of Miami, Miami, FL, USA.
³⁸ Department of Medicine & Pharmacology, Vanderbilt University Medical Center, Nashville, TN, USA.
³⁹ Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.
⁴⁰ Science 37, Los Angeles, CA, USA.
⁴¹ Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
⁴² Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
⁴³ Department of Dermatology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA.
⁴⁴ Departments of Dermatology and Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
⁴⁵ Department of Dermatology, University of South Florida, Cutaneous Oncology Program, Moffitt Cancer Center, Tampa, FL, USA.
⁴⁶ Department of Dermatology, Brigham & Women's Hospital, Harvard Medical School, Boston, MA, USA.
⁴⁷ Keck-USC School of Medicine, Los Angeles, CA, USA.
⁴⁸ Burn Surgery Service, Shriners Burns Hospital, Sumner Redstone Burn Center, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
⁴⁹ Division of Dermatology, Department of Internal Medicine, Ohio State University, Columbus, OH, USA.
⁵⁰ Department of Dermatology, University of California, San Francisco, CA, USA.
⁵¹ Department of Dermatology and Allergy, University Hospital of Munich, LMU, Munich, Germany.
⁵² Universit, EpiDermE, Créteil, France.

PMID: 33657677
DOI: 10.1111/bjd.19893

Abstract

Background: Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking.

Objectives: Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN.

Methods: Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method.

Results: Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements.

Conclusions: We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Child
Consensus
Humans
Research
Retrospective Studies
Stevens-Johnson Syndrome* / diagnosis
Stevens-Johnson Syndrome* / therapy