Development of a Skin-Directed Scoring System for Stevens-Johnson Syndrome and Epidermal Necrolysis: A Delphi Consensus Exercise.

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Citation: JAMA Dermatology. 159(7):772-777, 2023 Jul 01.PMID: 37256599Institution: MedStar Washington Hospital CenterDepartment: Burn CenterForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: *Stevens-Johnson Syndrome | Blister/pa [Pathology] | Consensus | Delphi Technique | Head | Humans | Skin/pa [Pathology] | Stevens-Johnson Syndrome/di [Diagnosis] | Year: 2023ISSN:
  • 2168-6068
Name of journal: JAMA dermatologyAbstract: Conclusions and Relevance: This consensus exercise confirmed the need for an EN skin-directed scoring system, nomenclature, and differentiation of specific morphologic traits, and identified the sites most affected. It also established a baseline consensus for a standardized EN instrument with consistent terminology.Evidence Review: A Delphi consensus using the RAND/UCLA appropriateness criteria was initiated with a core group from the Society of Dermatology Hospitalists to establish agreement on the optimal design for an EN cutaneous scoring instrument, terminology, morphologic traits, and sites of involvement.Findings: In round 1, the 54 participating dermatology hospitalists reached consensus on all 49 statements (30 appropriate, 3 inappropriate, 16 uncertain). In round 2, they agreed on another 15 statements (8 appropriate, 7 uncertain). There was consistent agreement on the need for a skin-specific instrument; on the most-often affected skin sites (head and neck, chest, upper back, ocular mucosa, oral mucosa); and that blanching erythema, dusky erythema, targetoid erythema, vesicles/bullae, desquamation, and erosions comprise the morphologic traits of EN and can be consistently differentiated.Importance: Scoring systems for Stevens-Johnson syndrome and epidermal necrolysis (EN) only estimate patient prognosis and are weighted toward comorbidities and systemic features; morphologic terminology for EN lesions is inconsistent.Objectives: To establish consensus among expert dermatologists on EN terminology, morphologic progression, and most-affected sites, and to build a framework for developing a skin-directed scoring system for EN.All authors: Albrecht J, Altman EM, Arakaki R, Ardern-Jones M, Beachkofsky TM, Bridges AG, Cardones AR, Chadha AA, Chen JK, Chen ST, Cheng K, Daveluy S, Davis MDP, DeNiro KL, Dobry A, Dominguez AR, Harp J, Kaffenberger BH, Keller JJ, King B, Korman AM, Kroshinsky D, Le ST, Lowenstein EJ, Luxenberg E, Mancuso JB, Markova A, Mauskar MM, Maverakis E, Micheletti RG, Milam P, Mostaghimi A, Motaparthi K, Nelson CA, Nguyen CV, Nutan F, Ortega-Loayza AG, Pasieka HB, Patel T, Rahnama-Moghadam S, Rekhtman S, Rojek NW, Rosenbach M, Sarihan M, Seminario-Vidal L, Shaigany S, Sharma TR, Shearer SM, Shields BE, Shinkai K, Strowd LC, Tartar DM, Thomas C, Trinidad J, Walls AC, Wanat KA, Waters M, Zaba LC, Ziemer CMFiscal year: FY2024Digital Object Identifier: Date added to catalog: 2023-08-15
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Conclusions and Relevance: This consensus exercise confirmed the need for an EN skin-directed scoring system, nomenclature, and differentiation of specific morphologic traits, and identified the sites most affected. It also established a baseline consensus for a standardized EN instrument with consistent terminology.

Evidence Review: A Delphi consensus using the RAND/UCLA appropriateness criteria was initiated with a core group from the Society of Dermatology Hospitalists to establish agreement on the optimal design for an EN cutaneous scoring instrument, terminology, morphologic traits, and sites of involvement.

Findings: In round 1, the 54 participating dermatology hospitalists reached consensus on all 49 statements (30 appropriate, 3 inappropriate, 16 uncertain). In round 2, they agreed on another 15 statements (8 appropriate, 7 uncertain). There was consistent agreement on the need for a skin-specific instrument; on the most-often affected skin sites (head and neck, chest, upper back, ocular mucosa, oral mucosa); and that blanching erythema, dusky erythema, targetoid erythema, vesicles/bullae, desquamation, and erosions comprise the morphologic traits of EN and can be consistently differentiated.

Importance: Scoring systems for Stevens-Johnson syndrome and epidermal necrolysis (EN) only estimate patient prognosis and are weighted toward comorbidities and systemic features; morphologic terminology for EN lesions is inconsistent.

Objectives: To establish consensus among expert dermatologists on EN terminology, morphologic progression, and most-affected sites, and to build a framework for developing a skin-directed scoring system for EN.

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