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

Waters M, Dobry A, Le ST, et al.  JAMA Dermatol. 2023;159(7):772–777. doi:10.1001/jamadermatol.2023.1347

Key Points

• Question  Is there a need for an EN skin-directed scoring system, consistent EN terminology, a better understanding of orphologic progression, and/or identification of the most-affected sites?
• Findings  This Delphi consensus exercise determined that there is a need for a skin-directed scoring system for EN with consensus achieved regarding terminology, morphologic traits, and most-affected locations of involvement.
• Meaning  The findings of this consensus exercise may be used as a framework for developing a skin-directed EN grading tool with the goal of precisely monitoring disease severity and changes during hospitalization.

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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