. 2023 Feb 22;18(1):33.

doi: 10.1186/s13023-023-02631-7.

Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus

S Ingen-Housz-Oro^#^{1

2

3

4}, V Schmidt^#^{5

6}, M M Ameri^{7

8

9}, R Abe¹⁰, A Brassard¹¹, A Mostaghimi¹², A S Paller¹³, A Romano¹⁴, B Didona^{15

16}, B H Kaffenberger^{15

17}, B Ben Said^{15

18

19}, B Y H Thong²⁰, B Ramsay²¹, E Brezinova^{15

22}, B Milpied^{15

18

23}, C G Mortz²⁴, C Y Chu²⁵, C Sotozono²⁶, J Gueudry^{18

27}, D G Fortune²⁸, S M Dridi^{18

29}, D Tartar³⁰, G Do-Pham^{18

31}, E Gabison³², E J Phillips^{33

34}, F Lewis³⁵, C Salavastru^{15

36}, B Horvath^{15

37}, J Dart³⁸, J Setterfield^{15

39}, J Newman⁴⁰, J T Schulz⁴¹, A Delcampe^{18

27

32

42}, K Brockow⁴³, L Seminario-Vidal⁴⁴, L Jörg^{45

46}, M P Watson⁴⁷, M Gonçalo⁴⁸, M Lucas^{49

50}, M Torres⁵¹, M H Noe⁵², N Hama¹⁰, N H Shear^{53

54}, P O'Reilly⁵⁵, P Wolkenstein^{56

15

18}, P Romanelli⁵⁷, R P Dodiuk-Gad^{58

59}, R G Micheletti⁶⁰, G S Tiplica^{15

61}, R Sheridan^{62

63

64}, S Rauz⁶⁵, S Ahmad³⁸, S L Chua^{15

66}, T H Flynn⁶⁷, W Pichler⁶⁸, S T Le¹¹, E Maverakis¹¹, S Walsh^{15

40}, L E French^#^{15

69

70}, M C Brüggen^{15

6

45

71}

Affiliations

¹ Department of Dermatology, AP-HP, Henri Mondor Hospital, 1 Rue Gustave Eiffel, 94000, Créteil, France. saskia.oro@aphp.fr.
² ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France. saskia.oro@aphp.fr.
³ Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France. saskia.oro@aphp.fr.
⁴ EpiDermE, Université Paris Est Créteil, Créteil, France. saskia.oro@aphp.fr.
⁵ University Hospital Basel, Basel, Switzerland.
⁶ Faculty of Medicine, University of Zurich, Zurich, Switzerland.
⁷ Faculty of Medicine, University of Zurich, Zurich, Switzerland. saskia.oro@aphp.fr.
⁸ Department of Dermatology, University Hospital Zurich, Zurich, Switzerland. saskia.oro@aphp.fr.
⁹ Christine Kühne-Center for Allergy Research and Education, Davos, Switzerland. saskia.oro@aphp.fr.
¹⁰ Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
¹¹ Department of Dermatology, UC Davis Medical Center, Sacramento, CA, USA.
¹² Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.
¹³ Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
¹⁴ Oasi Research Institute-IRCCS, Troina, Italy.
¹⁵ ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France.
¹⁶ Rare Disease Unit, I Dermatology Division, Istituto Dermopatico Dell'Immacolata, IRCCS, Rome, Italy.
¹⁷ The Ohio State University Wexner Medical Center Division of Dermatology, Upper Arlington, OH, USA.
¹⁸ Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France.
¹⁹ Department of Dermatology, CHU Edouard Herriot, Lyon, France.
²⁰ Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, Singapore.
²¹ Department of Dermatology, University Hospital Limerick, Limerick, Ireland.
²² First Department of Dermatovenereology, Masaryk University Faculty of Medicine, St. Ann's Faculty Hospital in Brno, Brno, Czech Republic.
²³ Department of Adult and Pediatric Dermatology, Bordeaux University Hospitals, Bordeaux, France.
²⁴ Department of Dermatology and Allergy Center, Odense Research Center for Anaphylaxis (ORCA), Odense University Hospital, Odense, Denmark.
²⁵ Department of Dermatology, National Taiwan University Hospital, National Taiwan University College of Medicine, No. 7, Chung-Shan South Road, Taipei, 10002, Taiwan.
²⁶ Department of Ophthalmology, Kyoto Prefectural University of Medicine, 465 Kajii-Cho, Hirokoji-Agaru, Kawaramach-Dori, Kamigyo-Ku, Kyoto, 602-0841, Japan.
²⁷ Department of Ophthalmology, CHU Charles-Nicolle, Rouen, France.
²⁸ Department of Psychology, University of Limerick, Limerick, Ireland.
²⁹ MICORALIS Laboratory, Department of Periodontology, Faculty of Dentistry, Côte d'Azur University, Saint Roch Hospital, Nice, France.
³⁰ Department of Dermatology, University of California Davis, Sacramento, CA, USA.
³¹ Department of Internal Medicine, Centre Hospitalier Intercommunal de Créteil, Créteil, France.
³² Fondation Ophtalmologique Adolphe de Rothschild, Paris, France.
³³ Institute for Immunology and Infectious Diseases, Murdoch University, Murdoch, WA, Australia.
³⁴ Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
³⁵ St John's Institute of Dermatology, Guy's and St Thomas' Hospital, London, UK.
³⁶ Department of Paediatric Dermatology, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
³⁷ Department of Dermatology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands.
³⁸ Moorfields Eye Hospital NHS Foundation Trust, The UCL Institute of Ophthalmology, London, UK.
³⁹ Department of Oral Medicine, Guy's and St Thomas' NHS Foundation Trust, London, UK.
⁴⁰ Department of Dermatology, King's College Hospital NHS Foundation Trust, London, UK.
⁴¹ Division of Burns, Massachusetts General Hospital, Boston, 02114, USA.
⁴² Department of Ophthalmology, CHU Bichat-Claude Bernard, Paris, France.
⁴³ Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.
⁴⁴ Department of Dermatology and Cutaneous Surgery, University of South Florida, Tampa, FL, USA.
⁴⁵ Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
⁴⁶ Division of Allergology and Clinical Immunology, Department of Pneumology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
⁴⁷ Cornea and External Eye Disease Service, Moorfields Eye Hospital, London, UK.
⁴⁸ Department of Dermatology, Coimbra University Hospital Center, Faculty of Medicine, University of Coimbra, Coimbra, Portugal.
⁴⁹ Medical School, University of Western Australia, Perth, WA, 6009, Australia.
⁵⁰ Department of Immunology, Sir Charles Gairdner Hospital, Pathwest Laboratory Medicine, Perth, WA, 6009, Australia.
⁵¹ Allergy Unit, IBIMA-Regional University Hospital of Malaga-UMA, Málaga, Spain.
⁵² Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
⁵³ Department of Dermatology, University of Toronto, Toronto, ON, Canada.
⁵⁴ Sunnybrook Health Sciences Centre, Toronto, ON, Canada.
⁵⁵ Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland.
⁵⁶ Department of Dermatology, AP-HP, Henri Mondor Hospital, 1 Rue Gustave Eiffel, 94000, Créteil, France.
⁵⁷ Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami, Miller School of Medicine, Miami, FL, USA.
⁵⁸ Dermatology Department, Emek Medical Center, Bruce Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel.
⁵⁹ Department of Medicine, University of Toronto, Toronto, Canada.
⁶⁰ Department of Dermatology and Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
⁶¹ 2Nd Department of Dermatology, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
⁶² Burn Service, Boston Shriners Hospital for Children, Boston, MA, USA.
⁶³ Division of Burns, Massachusetts General Hospital, Boston, MA, USA.
⁶⁴ Department of Surgery, Harvard Medical School, Boston, MA, USA.
⁶⁵ Academic Unit of Ophthalmology, Birmingham and Midland Eye Centre, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
⁶⁶ Department of Dermatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
⁶⁷ Ophthalmology, Bon Secours Hospital, Cork, Ireland.
⁶⁸ ADR-AC GmbH, Bern, Switzerland.
⁶⁹ Department of Dermatology, University Hospital, Munich University of Ludwig Maximilian, Munich, Germany.
⁷⁰ Dr Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
⁷¹ Christine Kühne-Center for Allergy Research and Education, Davos, Switzerland.

^# Contributed equally.

PMID: 36814255
PMCID: PMC9945700
DOI: 10.1186/s13023-023-02631-7

Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus

S Ingen-Housz-Oro et al. Orphanet J Rare Dis. 2023.

. 2023 Feb 22;18(1):33.

doi: 10.1186/s13023-023-02631-7.

Authors

Affiliations

¹ Department of Dermatology, AP-HP, Henri Mondor Hospital, 1 Rue Gustave Eiffel, 94000, Créteil, France. saskia.oro@aphp.fr.
² ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France. saskia.oro@aphp.fr.
³ Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France. saskia.oro@aphp.fr.
⁴ EpiDermE, Université Paris Est Créteil, Créteil, France. saskia.oro@aphp.fr.
⁵ University Hospital Basel, Basel, Switzerland.
⁶ Faculty of Medicine, University of Zurich, Zurich, Switzerland.
⁷ Faculty of Medicine, University of Zurich, Zurich, Switzerland. saskia.oro@aphp.fr.
⁸ Department of Dermatology, University Hospital Zurich, Zurich, Switzerland. saskia.oro@aphp.fr.
⁹ Christine Kühne-Center for Allergy Research and Education, Davos, Switzerland. saskia.oro@aphp.fr.
¹⁰ Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
¹¹ Department of Dermatology, UC Davis Medical Center, Sacramento, CA, USA.
¹² Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.
¹³ Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
¹⁴ Oasi Research Institute-IRCCS, Troina, Italy.
¹⁵ ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France.
¹⁶ Rare Disease Unit, I Dermatology Division, Istituto Dermopatico Dell'Immacolata, IRCCS, Rome, Italy.
¹⁷ The Ohio State University Wexner Medical Center Division of Dermatology, Upper Arlington, OH, USA.
¹⁸ Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France.
¹⁹ Department of Dermatology, CHU Edouard Herriot, Lyon, France.
²⁰ Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, Singapore.
²¹ Department of Dermatology, University Hospital Limerick, Limerick, Ireland.
²² First Department of Dermatovenereology, Masaryk University Faculty of Medicine, St. Ann's Faculty Hospital in Brno, Brno, Czech Republic.
²³ Department of Adult and Pediatric Dermatology, Bordeaux University Hospitals, Bordeaux, France.
²⁴ Department of Dermatology and Allergy Center, Odense Research Center for Anaphylaxis (ORCA), Odense University Hospital, Odense, Denmark.
²⁵ Department of Dermatology, National Taiwan University Hospital, National Taiwan University College of Medicine, No. 7, Chung-Shan South Road, Taipei, 10002, Taiwan.
²⁶ Department of Ophthalmology, Kyoto Prefectural University of Medicine, 465 Kajii-Cho, Hirokoji-Agaru, Kawaramach-Dori, Kamigyo-Ku, Kyoto, 602-0841, Japan.
²⁷ Department of Ophthalmology, CHU Charles-Nicolle, Rouen, France.
²⁸ Department of Psychology, University of Limerick, Limerick, Ireland.
²⁹ MICORALIS Laboratory, Department of Periodontology, Faculty of Dentistry, Côte d'Azur University, Saint Roch Hospital, Nice, France.
³⁰ Department of Dermatology, University of California Davis, Sacramento, CA, USA.
³¹ Department of Internal Medicine, Centre Hospitalier Intercommunal de Créteil, Créteil, France.
³² Fondation Ophtalmologique Adolphe de Rothschild, Paris, France.
³³ Institute for Immunology and Infectious Diseases, Murdoch University, Murdoch, WA, Australia.
³⁴ Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
³⁵ St John's Institute of Dermatology, Guy's and St Thomas' Hospital, London, UK.
³⁶ Department of Paediatric Dermatology, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
³⁷ Department of Dermatology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands.
³⁸ Moorfields Eye Hospital NHS Foundation Trust, The UCL Institute of Ophthalmology, London, UK.
³⁹ Department of Oral Medicine, Guy's and St Thomas' NHS Foundation Trust, London, UK.
⁴⁰ Department of Dermatology, King's College Hospital NHS Foundation Trust, London, UK.
⁴¹ Division of Burns, Massachusetts General Hospital, Boston, 02114, USA.
⁴² Department of Ophthalmology, CHU Bichat-Claude Bernard, Paris, France.
⁴³ Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany.
⁴⁴ Department of Dermatology and Cutaneous Surgery, University of South Florida, Tampa, FL, USA.
⁴⁵ Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
⁴⁶ Division of Allergology and Clinical Immunology, Department of Pneumology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
⁴⁷ Cornea and External Eye Disease Service, Moorfields Eye Hospital, London, UK.
⁴⁸ Department of Dermatology, Coimbra University Hospital Center, Faculty of Medicine, University of Coimbra, Coimbra, Portugal.
⁴⁹ Medical School, University of Western Australia, Perth, WA, 6009, Australia.
⁵⁰ Department of Immunology, Sir Charles Gairdner Hospital, Pathwest Laboratory Medicine, Perth, WA, 6009, Australia.
⁵¹ Allergy Unit, IBIMA-Regional University Hospital of Malaga-UMA, Málaga, Spain.
⁵² Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
⁵³ Department of Dermatology, University of Toronto, Toronto, ON, Canada.
⁵⁴ Sunnybrook Health Sciences Centre, Toronto, ON, Canada.
⁵⁵ Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland.
⁵⁶ Department of Dermatology, AP-HP, Henri Mondor Hospital, 1 Rue Gustave Eiffel, 94000, Créteil, France.
⁵⁷ Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami, Miller School of Medicine, Miami, FL, USA.
⁵⁸ Dermatology Department, Emek Medical Center, Bruce Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel.
⁵⁹ Department of Medicine, University of Toronto, Toronto, Canada.
⁶⁰ Department of Dermatology and Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
⁶¹ 2Nd Department of Dermatology, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
⁶² Burn Service, Boston Shriners Hospital for Children, Boston, MA, USA.
⁶³ Division of Burns, Massachusetts General Hospital, Boston, MA, USA.
⁶⁴ Department of Surgery, Harvard Medical School, Boston, MA, USA.
⁶⁵ Academic Unit of Ophthalmology, Birmingham and Midland Eye Centre, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
⁶⁶ Department of Dermatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
⁶⁷ Ophthalmology, Bon Secours Hospital, Cork, Ireland.
⁶⁸ ADR-AC GmbH, Bern, Switzerland.
⁶⁹ Department of Dermatology, University Hospital, Munich University of Ludwig Maximilian, Munich, Germany.
⁷⁰ Dr Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
⁷¹ Christine Kühne-Center for Allergy Research and Education, Davos, Switzerland.

^# Contributed equally.

PMID: 36814255
PMCID: PMC9945700
DOI: 10.1186/s13023-023-02631-7

Abstract

Background: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking.

Objectives: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae.

Methods: Participants were sent a survey via the online tool "Survey Monkey" consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method.

Results: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as 'appropriate'; four statements were considered 'uncertain', and ultimately finally discarded.

Conclusions: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.

Keywords: Consensus; Delphi; Epidermal necrolysis; Quality of life; Sequelae; Stevens-Johnson syndrome; Toxic epidermal necrolysis.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

**Fig. 1**
Top: Flow chart of the experts. Bottom: Flow chart of the statements

See this image and copyright information in PMC

References

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Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus

Affiliations

Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources