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

• Authors: INGEN-HOUSZ-ORO S.; SCHMIDT V.; AMERI M. M.; ABE R.; BRASSARD A.; MOSTAGHIMI A.; PALLER A. S.; ROMANO A.; DIDONA B.; KAFFENBERGER B. H.; BEN SAID B.; THONG B. Y. H.; RAMSAY B.; BŘEZINOVÁ Eva; MILPIED B.; MORTZ C. G.; CHU C. Y.; SOTOZONO C.; GUEUDRY J.; FORTUNE D. G.; DRIDI S. M.; TARTAR D.; DO-PHAM G.; GABISON E.; PHILLIPS E. J.; LEWIS F.; SALAVASTRU C.; HORVATH B.; DART J.; SETTERFIELD J.; NEWMAN J.; SCHULZ J. T.; DELCAMPE A.; BROCKOW K.; SEMINARIO-VIDAL L.; JOERG L.; WATSON M. P.; GONCALO M.; LUCAS M.; TORRES M.; NOE M. H.; HAMA N.; SHEAR N. H.; REILLY P.; WOLKENSTEIN P.; ROMANELLI P.; DODIUK-GAD R. P.; MICHELETTI R. G.; TIPLICA G. S.; SHERIDAN R.; RAUZ S.; AHMAD S.; CHUA S. L.; FLYNN T. H.; PICHLER W.; LE S. T.; MAVERAKIS E.; WALSH S.; FRENCH L. E.; BRUGGEN M. C.
• Year of publication: 2023
• Type: Article in Periodical
• Magazine / Source: Orphanet Journal of Rare Diseases
• MU Faculty or unit: Faculty of Medicine
• web: https://ojrd.biomedcentral.com/articles/10.1186/s13023-023-02631-7
• Doi: http://dx.doi.org/10.1186/s13023-023-02631-7
• Keywords: Epidermal necrolysis; Stevens-Johnson syndrome; Toxic epidermal necrolysis; Sequelae; Quality of life; Delphi; Consensus
• Description: BackgroundLong-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.ObjectivesWe conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae.MethodsParticipants 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.ResultsFifty-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.ConclusionsOur DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.