Development of a skin-directed scoring system for Stevens-Johnson Syndrome and epidermal necrolysis: A Delphi consensus exercise Journal Article


Authors: Waters, M.; Dobry, A.; Le, S. T.; Shinkai, K.; Beachkofsky, T. M.; Davis, M. D. P.; Dominguez, A. R.; Kroshinsky, D.; Markova, A.; Micheletti, R. G.; Mostaghimi, A.; Pasieka, H. B.; Rosenbach, M.; Seminario-Vidal, L.; Trinidad, J.; Albrecht, J.; Altman, E. M.; Arakaki, R.; Ardern-Jones, M.; Bridges, A. G.; Cardones, A. R.; Chadha, A. A.; Chen, J. K.; Chen, S. T.; Cheng, K.; Daveluy, S.; DeNiro, K. L.; Harp, J.; Keller, J. J.; King, B.; Korman, A. M.; Lowenstein, E. J.; Luxenberg, E.; Mancuso, J. B.; Mauskar, M. M.; Milam, P.; Motaparthi, K.; Nelson, C. A.; Nguyen, C. V.; Nutan, F.; Ortega-Loayza, A. G.; Patel, T.; Rahnama-Moghadam, S.; Rekhtman, S.; Rojek, N. W.; Sarihan, M.; Shaigany, S.; Sharma, T. R.; Shearer, S. M.; Shields, B. E.; Strowd, L. C.; Tartar, D. M.; Thomas, C.; Wanat, K. A.; Walls, A. C.; Zaba, L. C.; Ziemer, C. M.; Maverakis, E.; Kaffenberger, B. H.
Article Title: Development of a skin-directed scoring system for Stevens-Johnson Syndrome and epidermal necrolysis: A Delphi consensus exercise
Abstract: 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. 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. 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.
Keywords: consensus; pathology; skin; delphi study; delphi technique; stevens johnson syndrome; head; blister; stevens-johnson syndrome; humans; human
Journal Title: JAMA Dermatology
Volume: 159
Issue: 7
ISSN: 2168-6068
Publisher: American Medical Association  
Date Published: 2023-06-01
Start Page: 772
End Page: 777
Language: English
DOI: 10.1001/jamadermatol.2023.1347
PUBMED: 37256599
PROVIDER: scopus
PMCID: PMC10838134
DOI/URL:
Notes: The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PDF -- Erratum issued, see DOI: 10.1001/jamadermatol.2023.2502 -- Source: Scopus
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  1. Alina Markova
    87 Markova