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Ingen-Housz-Oro S, Schmidt V, Ameri MM, Abe R, Brassard A, Mostaghimi A, Paller AS, Romano A, Didona B, Kaffenberger BH, Ben Said B, Thong BYH, Ramsay B, Brezinova E, Milpied B, Mortz CG, Chu CY, Sotozono C, Gueudry J, Fortune DG, Dridi SM, Tartar D, Do-Pham G, Gabison E, Phillips EJ, Lewis F, Salavastru C, Horvath B, Dart J, Setterfield J, Newman J, Schulz JT, Delcampe A, Brockow K, Seminario-Vidal L, Jörg L, Watson MP, Gonçalo M, Lucas M, Torres M, Noe MH, Hama N, Shear NH, O’Reilly P, Wolkenstein P, Romanelli P, Dodiuk-Gad RP, Micheletti RG, Tiplica GS, Sheridan R, Rauz S, Ahmad S, Chua SL, Flynn TH, Pichler W, Le ST, Maverakis E, Walsh S, French LE, Brüggen MC. Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus. Orphanet J Rare Dis 2023; 18:33. [PMID: 36814255 PMCID: PMC9945700 DOI: 10.1186/s13023-023-02631-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2022] [Accepted: 02/06/2023] [Indexed: 02/24/2023] Open
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.
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Affiliation(s)
- S. Ingen-Housz-Oro
- grid.412116.10000 0004 1799 3934Department of Dermatology, AP-HP, Henri Mondor Hospital, 1 Rue Gustave Eiffel, 94000 Créteil, France ,ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France ,grid.410511.00000 0001 2149 7878EpiDermE, Université Paris Est Créteil, Créteil, France
| | - V. Schmidt
- grid.410567.1University Hospital Basel, Basel, Switzerland ,grid.7400.30000 0004 1937 0650Faculty of Medicine, University of Zurich, Zurich, Switzerland
| | - M. M. Ameri
- grid.7400.30000 0004 1937 0650Faculty of Medicine, University of Zurich, Zurich, Switzerland ,grid.412004.30000 0004 0478 9977Department of Dermatology, University Hospital Zurich, Zurich, Switzerland ,grid.507894.70000 0004 4700 6354Christine Kühne-Center for Allergy Research and Education, Davos, Switzerland
| | - R. Abe
- grid.260975.f0000 0001 0671 5144Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
| | - A. Brassard
- grid.413079.80000 0000 9752 8549Department of Dermatology, UC Davis Medical Center, Sacramento, CA USA
| | - A. Mostaghimi
- grid.62560.370000 0004 0378 8294Department of Dermatology, Brigham and Women’s Hospital, Boston, MA USA
| | - A. S. Paller
- grid.16753.360000 0001 2299 3507Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, IL USA
| | - A. Romano
- grid.419843.30000 0001 1250 7659Oasi Research Institute-IRCCS, Troina, Italy
| | - B. Didona
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.419457.a0000 0004 1758 0179Rare Disease Unit, I Dermatology Division, Istituto Dermopatico Dell’Immacolata, IRCCS, Rome, Italy
| | - B. H. Kaffenberger
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.412332.50000 0001 1545 0811The Ohio State University Wexner Medical Center Division of Dermatology, Upper Arlington, OH USA
| | - B. Ben Said
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France ,Department of Dermatology, CHU Edouard Herriot, Lyon, France
| | - B. Y. H. Thong
- grid.240988.f0000 0001 0298 8161Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, Singapore
| | - B. Ramsay
- grid.415522.50000 0004 0617 6840Department of Dermatology, University Hospital Limerick, Limerick, Ireland
| | - E. Brezinova
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.10267.320000 0001 2194 0956First Department of Dermatovenereology, Masaryk University Faculty of Medicine, St. Ann’s Faculty Hospital in Brno, Brno, Czech Republic
| | - B. Milpied
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France ,grid.412041.20000 0001 2106 639XDepartment of Adult and Pediatric Dermatology, Bordeaux University Hospitals, Bordeaux, France
| | - C. G. Mortz
- grid.7143.10000 0004 0512 5013Department of Dermatology and Allergy Center, Odense Research Center for Anaphylaxis (ORCA), Odense University Hospital, Odense, Denmark
| | - C. Y. Chu
- grid.19188.390000 0004 0546 0241Department of Dermatology, National Taiwan University Hospital, National Taiwan University College of Medicine, No. 7, Chung-Shan South Road, Taipei, 10002 Taiwan
| | - C. Sotozono
- grid.272458.e0000 0001 0667 4960Department of Ophthalmology, Kyoto Prefectural University of Medicine, 465 Kajii-Cho, Hirokoji-Agaru, Kawaramach-Dori, Kamigyo-Ku, Kyoto, 602-0841 Japan
| | - J. Gueudry
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France ,grid.417615.0Department of Ophthalmology, CHU Charles-Nicolle, Rouen, France
| | - D. G. Fortune
- grid.10049.3c0000 0004 1936 9692Department of Psychology, University of Limerick, Limerick, Ireland
| | - S. M. Dridi
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France ,grid.416670.2MICORALIS Laboratory, Department of Periodontology, Faculty of Dentistry, Côte d’Azur University, Saint Roch Hospital, Nice, France
| | - D. Tartar
- grid.27860.3b0000 0004 1936 9684Department of Dermatology, University of California Davis, Sacramento, CA USA
| | - G. Do-Pham
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France ,grid.414145.10000 0004 1765 2136Department of Internal Medicine, Centre Hospitalier Intercommunal de Créteil, Créteil, France
| | - E. Gabison
- grid.417888.a0000 0001 2177 525XFondation Ophtalmologique Adolphe de Rothschild, Paris, France
| | - E. J. Phillips
- grid.1025.60000 0004 0436 6763Institute for Immunology and Infectious Diseases, Murdoch University, Murdoch, WA Australia ,grid.412807.80000 0004 1936 9916Department of Medicine, Vanderbilt University Medical Center, Nashville, TN USA
| | - F. Lewis
- grid.425213.3St John’s Institute of Dermatology, Guy’s and St Thomas’ Hospital, London, UK
| | - C. Salavastru
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,Department of Paediatric Dermatology, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
| | - B. Horvath
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.4830.f0000 0004 0407 1981Department of Dermatology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB Groningen, The Netherlands
| | - J. Dart
- grid.83440.3b0000000121901201Moorfields Eye Hospital NHS Foundation Trust, The UCL Institute of Ophthalmology, London, UK
| | - J. Setterfield
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.420545.20000 0004 0489 3985Department of Oral Medicine, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
| | - J. Newman
- grid.429705.d0000 0004 0489 4320Department of Dermatology, King’s College Hospital NHS Foundation Trust, London, UK
| | - J. T. Schulz
- grid.32224.350000 0004 0386 9924Division of Burns, Massachusetts General Hospital, Boston, 02114 USA
| | - A. Delcampe
- Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France ,grid.417615.0Department of Ophthalmology, CHU Charles-Nicolle, Rouen, France ,grid.417888.a0000 0001 2177 525XFondation Ophtalmologique Adolphe de Rothschild, Paris, France ,grid.411119.d0000 0000 8588 831XDepartment of Ophthalmology, CHU Bichat-Claude Bernard, Paris, France
| | - K. Brockow
- grid.6936.a0000000123222966Department of Dermatology and Allergy Biederstein, School of Medicine, Technical University of Munich, Munich, Germany
| | - L. Seminario-Vidal
- grid.170693.a0000 0001 2353 285XDepartment of Dermatology and Cutaneous Surgery, University of South Florida, Tampa, FL USA
| | - L. Jörg
- grid.412004.30000 0004 0478 9977Department of Dermatology, University Hospital Zurich, Zurich, Switzerland ,grid.5734.50000 0001 0726 5157Division of Allergology and Clinical Immunology, Department of Pneumology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - M. P. Watson
- grid.439257.e0000 0000 8726 5837Cornea and External Eye Disease Service, Moorfields Eye Hospital, London, UK
| | - M. Gonçalo
- grid.28911.330000000106861985Department of Dermatology, Coimbra University Hospital Center, Faculty of Medicine, University of Coimbra, Coimbra, Portugal
| | - M. Lucas
- grid.1012.20000 0004 1936 7910Medical School, University of Western Australia, Perth, WA 6009 Australia ,grid.3521.50000 0004 0437 5942Department of Immunology, Sir Charles Gairdner Hospital, Pathwest Laboratory Medicine, Perth, WA 6009 Australia
| | - M. Torres
- grid.452525.1Allergy Unit, IBIMA-Regional University Hospital of Malaga-UMA, Málaga, Spain
| | - M. H. Noe
- grid.62560.370000 0004 0378 8294Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA USA
| | - N. Hama
- grid.260975.f0000 0001 0671 5144Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
| | - N. H. Shear
- grid.17063.330000 0001 2157 2938Department of Dermatology, University of Toronto, Toronto, ON Canada ,grid.413104.30000 0000 9743 1587Sunnybrook Health Sciences Centre, Toronto, ON Canada
| | - P. O’Reilly
- grid.10049.3c0000 0004 1936 9692Department of Nursing and Midwifery, University of Limerick, Limerick, Ireland
| | - P. Wolkenstein
- grid.412116.10000 0004 1799 3934Department of Dermatology, AP-HP, Henri Mondor Hospital, 1 Rue Gustave Eiffel, 94000 Créteil, France ,ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,Reference Center for Toxic Bullous Dermatoses and Severe Drug Reactions TOXIBUL, Créteil, France
| | - P. Romanelli
- grid.26790.3a0000 0004 1936 8606Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami, Miller School of Medicine, Miami, FL USA
| | - R. P. Dodiuk-Gad
- grid.6451.60000000121102151Dermatology Department, Emek Medical Center, Bruce Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel ,grid.17063.330000 0001 2157 2938Department of Medicine, University of Toronto, Toronto, Canada
| | - R. G. Micheletti
- grid.25879.310000 0004 1936 8972Department of Dermatology and Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA USA
| | - G. S. Tiplica
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,2Nd Department of Dermatology, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania
| | - R. Sheridan
- grid.415829.30000 0004 0449 5362Burn Service, Boston Shriners Hospital for Children, Boston, MA USA ,grid.32224.350000 0004 0386 9924Division of Burns, Massachusetts General Hospital, Boston, MA USA ,grid.38142.3c000000041936754XDepartment of Surgery, Harvard Medical School, Boston, MA USA
| | - S. Rauz
- grid.6572.60000 0004 1936 7486Academic Unit of Ophthalmology, Birmingham and Midland Eye Centre, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK
| | - S. Ahmad
- grid.83440.3b0000000121901201Moorfields Eye Hospital NHS Foundation Trust, The UCL Institute of Ophthalmology, London, UK
| | - S. L. Chua
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.412563.70000 0004 0376 6589Department of Dermatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - T. H. Flynn
- grid.460892.10000 0004 0389 5639Ophthalmology, Bon Secours Hospital, Cork, Ireland
| | - W. Pichler
- grid.482939.dADR-AC GmbH, Bern, Switzerland
| | - S. T. Le
- grid.413079.80000 0000 9752 8549Department of Dermatology, UC Davis Medical Center, Sacramento, CA USA
| | - E. Maverakis
- grid.413079.80000 0000 9752 8549Department of Dermatology, UC Davis Medical Center, Sacramento, CA USA
| | - S. Walsh
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.429705.d0000 0004 0489 4320Department of Dermatology, King’s College Hospital NHS Foundation Trust, London, UK
| | - L. E. French
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.411095.80000 0004 0477 2585Department of Dermatology, University Hospital, Munich University of Ludwig Maximilian, Munich, Germany ,grid.26790.3a0000 0004 1936 8606Dr Phillip Frost Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL USA
| | - M. C. Brüggen
- ToxiTEN Group, European Reference Network for Rare Skin Diseases, Paris, France ,grid.7400.30000 0004 1937 0650Faculty of Medicine, University of Zurich, Zurich, Switzerland ,grid.412004.30000 0004 0478 9977Department of Dermatology, University Hospital Zurich, Zurich, Switzerland ,grid.507894.70000 0004 4700 6354Christine Kühne-Center for Allergy Research and Education, Davos, Switzerland
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Ingen-Housz-Oro S, Duong TA, Bensaid B, Bellon N, de Prost N, Lu D, Lebrun-Vignes B, Gueudry J, Bequignon E, Zaghbib K, Royer G, Colin A, Do-Pham G, Bodemer C, Ortonne N, Barbaud A, Fardet L, Chosidow O, Wolkenstein P. Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins). Orphanet J Rare Dis 2018; 13:56. [PMID: 29636107 PMCID: PMC5894129 DOI: 10.1186/s13023-018-0793-7] [Citation(s) in RCA: 39] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2017] [Accepted: 03/22/2018] [Indexed: 12/18/2022] Open
Abstract
Epidermal necrolysis (EN) encompasses Stevens-Johnson syndrome (SJS, < 10% of the skin affected), Lyell syndrome (toxic epidermal necrolysis, TEN, with ≥30% of the skin affected) and an overlap syndrome (10 to 29% of the skin affected). These rare diseases are caused, in 85% of cases, by pharmacological treatments, with symptoms occurring 4 to 28 days after treatment initiation. Mortality is 20 to 25% during the acute phase, and almost all patients display disabling sequelae (mostly ocular impairment and psychological distress). The objective of this French national diagnosis and care protocol (protocole national de diagnostic et de soins; PNDS), based on a critical literature review and on a multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with EN. This PNDS, written by the French National Reference Center for Toxic Bullous Dermatoses was updated in 2017 (https://www.has-sante.fr/portail/jcms/c_1012735/fr/necrolyse-epidermique-syndromes-de-stevens-johnson-et-de-lyell). The cornerstone of the management of these patients during the acute phase is an immediate withdrawal of the responsible drug, patient management in a dermatology department, intensive care or burn units used to dealing with this disease, supportive care and close monitoring, the prevention and treatment of infections, and a multidisciplinary approach to sequelae. Based on published data, it is not currently possible to recommend any specific immunomodulatory treatment. Only the culprit drug and chemically similar molecules must be lifelong contraindicated.
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Affiliation(s)
- Saskia Ingen-Housz-Oro
- Dermatology Department, AP-HP, Henri Mondor Hospital, 51 avenue du maréchal de Lattre de Tassigny, 94000, Créteil, France. .,French National Reference Center for Toxic Bullous Dermatoses, Créteil, France. .,EA7379 EpiDermE (Epidemiologie en Dermatologie et Evaluation des Thérapeutiques), Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France.
| | - Tu-Anh Duong
- Dermatology Department, AP-HP, Henri Mondor Hospital, 51 avenue du maréchal de Lattre de Tassigny, 94000, Créteil, France.,French National Reference Center for Toxic Bullous Dermatoses, Créteil, France
| | - Benoit Bensaid
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Dermatology Department, Edouard Herriot Hospital, Lyon, France
| | - Nathalia Bellon
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Dermatology Department, AP-HP, Necker Hospital, Paris, France
| | - Nicolas de Prost
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Intensive Care Unit, AP-HP, Henri Mondor Hospital, Créteil, France.,Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France
| | - Dévy Lu
- Dermatology Department, AP-HP, Henri Mondor Hospital, 51 avenue du maréchal de Lattre de Tassigny, 94000, Créteil, France.,French National Reference Center for Toxic Bullous Dermatoses, Créteil, France
| | - Bénédicte Lebrun-Vignes
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Pharmacovigilance Department, AP-HP, La Pitié Salpêtrière Hospital, Paris, France
| | - Julie Gueudry
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Ophthalmology Department, Charles Nicolle Hospital, Rouen, France
| | - Emilie Bequignon
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Ear Nose and Throat Department, AP-HP, Henri Mondor Hospital, Créteil, France
| | - Karim Zaghbib
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Psychiatry Department, AP-HP, Henri Mondor Hospital, Créteil, France
| | - Gérard Royer
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Ophthalmology Department, AP-HP, Henri Mondor Hospital, Créteil, France
| | - Audrey Colin
- Dermatology Department, AP-HP, Henri Mondor Hospital, 51 avenue du maréchal de Lattre de Tassigny, 94000, Créteil, France.,French National Reference Center for Toxic Bullous Dermatoses, Créteil, France
| | - Giao Do-Pham
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Dermatology Department, Centre Hospitalier Intercommunal de Créteil, Créteil, France
| | - Christine Bodemer
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Dermatology Department, AP-HP, Necker Hospital, Paris, France
| | - Nicolas Ortonne
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Pathology Department, AP-HP, Henri Mondor Hospital, Créteil, France.,Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France
| | - Annick Barbaud
- French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,Dermatology Department, AP-HP, Tenon Hospital, Paris, France
| | - Laurence Fardet
- Dermatology Department, AP-HP, Henri Mondor Hospital, 51 avenue du maréchal de Lattre de Tassigny, 94000, Créteil, France.,French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,EA7379 EpiDermE (Epidemiologie en Dermatologie et Evaluation des Thérapeutiques), Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France
| | - Olivier Chosidow
- Dermatology Department, AP-HP, Henri Mondor Hospital, 51 avenue du maréchal de Lattre de Tassigny, 94000, Créteil, France.,French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,EA7379 EpiDermE (Epidemiologie en Dermatologie et Evaluation des Thérapeutiques), Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France.,Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France
| | - Pierre Wolkenstein
- Dermatology Department, AP-HP, Henri Mondor Hospital, 51 avenue du maréchal de Lattre de Tassigny, 94000, Créteil, France.,French National Reference Center for Toxic Bullous Dermatoses, Créteil, France.,EA7379 EpiDermE (Epidemiologie en Dermatologie et Evaluation des Thérapeutiques), Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France.,Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France
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