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

Authors

Margo Waters
Allison Dobry
Stephanie T Le
Kanade Shinkai
Thomas M Beachkofsky
Mark D P Davis
Arturo R Dominguez
Daniela Kroshinsky
Alina Markova
Steven Daveluy, Beaumont Health

Document Type

Article

Publication Date

7-1-2023

Publication Title

JAMA Dermatology

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.

Volume

159

Issue

7

First Page

772

Last Page

777

Recommended Citation

Waters M, Dobry A, Le ST, Shinkai K, Beachkofsky TM, Davis MDP, et al. [Daveluy S]. Development of a skin-directed scoring system for Stevens-Johnson syndrome and epidermal necrolysis: a delphi consensus exercise. JAMA Dermatol. 2023 Jul 1;159(7):772-777. doi: 10.1001/jamadermatol.2023.1347. Erratum in: JAMA Dermatol. 2023 Jul 1;159(7):793. PMID: 37256599.

DOI

10.1001/jamadermatol.2023.1347

ISSN

2168-6084

PubMed ID

37256599