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. 2023 Jul 1;159(7):772-777.
doi: 10.1001/jamadermatol.2023.1347.

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

Margo Waters  1 , Allison Dobry  2 , Stephanie T Le  3 , Kanade Shinkai  2 , Thomas M Beachkofsky  4 , Mark D P Davis  5 , Arturo R Dominguez  6 , Daniela Kroshinsky  7 , Alina Markova  8 , Robert G Micheletti  9 , Arash Mostaghimi  10 , Helena B Pasieka  11   12 , Misha Rosenbach  9 , Lucia Seminario-Vidal  13 , John Trinidad  7 , Joerg Albrecht  14 , Emily M Altman  15 , Ryan Arakaki  2 , Michael Ardern-Jones  16 , Alina G Bridges  17 , Adela R Cardones  18 , Angad A Chadha  19 , Jennifer K Chen  20 , Steven T Chen  7 , Kyle Cheng  21 , Steven Daveluy  22 , Katherine L DeNiro  23 , Joanna Harp  24 , Jesse J Keller  25 , Brett King  26 , Abraham M Korman  27 , Eve J Lowenstein  28 , Erin Luxenberg  29 , Jennifer Brescoll Mancuso  30 , Melissa M Mauskar  6 , Philip Milam  31 , Kiran Motaparthi  32 , Caroline A Nelson  26 , Cuong V Nguyen  33 , Fnu Nutan  34 , Alex G Ortega-Loayza  25 , Tejesh Patel  35 , Sahand Rahnama-Moghadam  36 , Sergey Rekhtman  17 , Nathan W Rojek  37 , Mansi Sarihan  38 , Sheila Shaigany  17 , Timmie R Sharma  39 , Sabrina M Shearer  18 , Bridget E Shields  40 , Lindsay C Strowd  41 , Danielle M Tartar  3 , Cristina Thomas  6 , Karolyn A Wanat  42 , Andrew C Walls  10 , Lisa C Zaba  20 , Carolyn M Ziemer  43 , Emanual Maverakis  3 , Benjamin H Kaffenberger  27
Affiliations

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

Margo Waters et al. JAMA Dermatol. .

Erratum in

  • Error in Author Surname in Byline.
    [No authors listed] [No authors listed] JAMA Dermatol. 2023 Jul 1;159(7):793. doi: 10.1001/jamadermatol.2023.2502. JAMA Dermatol. 2023. PMID: 37466714 Free PMC article. No abstract available.

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.

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Conflict of interest statement

Conflicts of interest: B.H.K. receives clinical trials funding from Onquality, AnaptysBio, Biogen, and Bristol-Myers-Squibb. He is a consultant for Eli Lilly Co. and is funded by the Dermatology Foundation.

A.G.O.L. has served in advisory boards for Janssen, BMS, and Boeheringer Ingelheim, and as a consultant for Genentech and Guidepoint. He has also received research grants from Eli Lilly Co, OHSU SOM Gerlinger research award, and Medical Research Foundation of Oregon.

L.S.V. currently is an employee and shareholder of Eli Lily and Company. L.S.V had advisory board relationships with Novartis, Boehringer Ingelheim, Helsinn, Kyowa Kirin, Regeneron, Blueprint. She or her institution received research funding from Eli Lilly and Company, Soligenix, Helsinn, Eisai, Boehringer Ingelheim, Novartis, AbbVie, BMS, Celgene, Glenmark, Kyowa Kirin, Amgen, AnaptysBio, Innate Pharma. L.S.V. has been a speaker for Helsinn, Kyowa Kirin.

A.M. serves as a consultant for Alira Health Ventures and Blueprint Medicines. She has received research funding from Incyte Corp and Amryt Pharma and royalties from UpToDate.

B.E.S. is an Assistant Section Editor for JAMA Dermatology.

S.T.C. serves as a consultant for Pfizer, Novartis, Scholar Rock, and VisualDx. He has received honoraria from Pfizer for work on an advisory board for digital media.

M.R. is a consultant for Merck, Janssen, Novartis, Processa, and Eli Lilly. He receives research funding from Processa.

All other authors declare that they have no relevant conflicts of interest.

Figures

Figure 1:
Figure 1:
Flowchart illustrating the work steps of the Delphi exercise
See this image and copyright information in PMC

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