Orphanet Journal of Rare Diseases (Feb 2023)
Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus
- S. Ingen-Housz-Oro,
- V. Schmidt,
- M. M. Ameri,
- R. Abe,
- A. Brassard,
- A. Mostaghimi,
- A. S. Paller,
- A. Romano,
- B. Didona,
- B. H. Kaffenberger,
- B. Ben Said,
- B. Y. H. Thong,
- B. Ramsay,
- E. Brezinova,
- B. Milpied,
- C. G. Mortz,
- C. Y. Chu,
- C. Sotozono,
- J. Gueudry,
- D. G. Fortune,
- S. M. Dridi,
- D. Tartar,
- G. Do-Pham,
- E. Gabison,
- E. J. Phillips,
- F. Lewis,
- C. Salavastru,
- B. Horvath,
- J. Dart,
- J. Setterfield,
- J. Newman,
- J. T. Schulz,
- A. Delcampe,
- K. Brockow,
- L. Seminario-Vidal,
- L. Jörg,
- M. P. Watson,
- M. Gonçalo,
- M. Lucas,
- M. Torres,
- M. H. Noe,
- N. Hama,
- N. H. Shear,
- P. O’Reilly,
- P. Wolkenstein,
- P. Romanelli,
- R. P. Dodiuk-Gad,
- R. G. Micheletti,
- G. S. Tiplica,
- R. Sheridan,
- S. Rauz,
- S. Ahmad,
- S. L. Chua,
- T. H. Flynn,
- W. Pichler,
- S. T. Le,
- E. Maverakis,
- S. Walsh,
- L. E. French,
- M. C. Brüggen
Affiliations
- S. Ingen-Housz-Oro
- Department of Dermatology, AP-HP, Henri Mondor Hospital
- V. Schmidt
- University Hospital Basel
- M. M. Ameri
- Faculty of Medicine, University of Zurich
- R. Abe
- Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences
- A. Brassard
- Department of Dermatology, UC Davis Medical Center
- A. Mostaghimi
- Department of Dermatology, Brigham and Women’s Hospital
- A. S. Paller
- Department of Dermatology, Northwestern University Feinberg School of Medicine
- A. Romano
- Oasi Research Institute-IRCCS
- B. Didona
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- B. H. Kaffenberger
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- B. Ben Said
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- B. Y. H. Thong
- Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital
- B. Ramsay
- Department of Dermatology, University Hospital Limerick
- E. Brezinova
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- B. Milpied
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- C. G. Mortz
- Department of Dermatology and Allergy Center, Odense Research Center for Anaphylaxis (ORCA), Odense University Hospital
- C. Y. Chu
- Department of Dermatology, National Taiwan University Hospital, National Taiwan University College of Medicine
- C. Sotozono
- Department of Ophthalmology, Kyoto Prefectural University of Medicine
- J. Gueudry
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL
- D. G. Fortune
- Department of Psychology, University of Limerick
- S. M. Dridi
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL
- D. Tartar
- Department of Dermatology, University of California Davis
- G. Do-Pham
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL
- E. Gabison
- Fondation Ophtalmologique Adolphe de Rothschild
- E. J. Phillips
- Institute for Immunology and Infectious Diseases, Murdoch University
- F. Lewis
- St John’s Institute of Dermatology, Guy’s and St Thomas’ Hospital
- C. Salavastru
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- B. Horvath
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- J. Dart
- Moorfields Eye Hospital NHS Foundation Trust, The UCL Institute of Ophthalmology
- J. Setterfield
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- J. Newman
- Department of Dermatology, King’s College Hospital NHS Foundation Trust
- J. T. Schulz
- Division of Burns, Massachusetts General Hospital
- A. Delcampe
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL
- K. Brockow
- Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich
- L. Seminario-Vidal
- Department of Dermatology and Cutaneous Surgery, University of South Florida
- L. Jörg
- Department of Dermatology, University Hospital Zurich
- M. P. Watson
- Cornea and External Eye Disease Service, Moorfields Eye Hospital
- M. Gonçalo
- Department of Dermatology, Coimbra University Hospital Center, Faculty of Medicine, University of Coimbra
- M. Lucas
- Medical School, University of Western Australia
- M. Torres
- Allergy Unit, IBIMA-Regional University Hospital of Malaga-UMA
- M. H. Noe
- Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School
- N. Hama
- Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences
- N. H. Shear
- Department of Dermatology, University of Toronto
- P. O’Reilly
- Department of Nursing and Midwifery, University of Limerick
- P. Wolkenstein
- Department of Dermatology, AP-HP, Henri Mondor Hospital
- P. Romanelli
- Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami, Miller School of Medicine
- R. P. Dodiuk-Gad
- Dermatology Department, Emek Medical Center, Bruce Rappaport Faculty of Medicine, Technion - Institute of Technology
- R. G. Micheletti
- Department of Dermatology and Medicine, Perelman School of Medicine, University of Pennsylvania
- G. S. Tiplica
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- R. Sheridan
- Burn Service, Boston Shriners Hospital for Children
- S. Rauz
- Academic Unit of Ophthalmology, Birmingham and Midland Eye Centre, Institute of Inflammation and Ageing, University of Birmingham
- S. Ahmad
- Moorfields Eye Hospital NHS Foundation Trust, The UCL Institute of Ophthalmology
- S. L. Chua
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- T. H. Flynn
- Ophthalmology, Bon Secours Hospital
- W. Pichler
- ADR-AC GmbH
- S. T. Le
- Department of Dermatology, UC Davis Medical Center
- E. Maverakis
- Department of Dermatology, UC Davis Medical Center
- S. Walsh
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- L. E. French
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- M. C. Brüggen
- ToxiTEN Group, European Reference Network for Rare Skin Diseases
- DOI
- https://doi.org/10.1186/s13023-023-02631-7
- Journal volume & issue
-
Vol. 18,
no. 1
pp. 1 – 10
Abstract
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
- Epidermal necrolysis
- Stevens-Johnson syndrome
- Toxic epidermal necrolysis
- Sequelae
- Quality of life
- Delphi
