COLUMBUS, OH — Stevens-Johnson syndrome/toxic epidermal necrolysis (EN) is a severe adverse mucocutaneous drug reaction with life-threatening implications, usually caused by drug reactions.
A recent study in JAMA Dermatology pointed out that prognostic models, such as the SCORTEN (severity-of-illness score for toxic epidermal necrolysis) and the ABCD-10 (age, bicarbonate, cancer, dialysis, 10% body surface area), are calculated within 24 hours of admission to assess risk of mortality.1
“Although both scores have excellent discriminatory power, the SCORTEN and ABCD-10 are static assessments of survival and not intended to assess cutaneous disease progression or improvement,” wrote researchers led by The Ohio State University College of Medicine and including participation from the James A. Haley Veterans Hospital in Tampa, FL, and the Uniformed Services University in Bethesda, MD. “Recognizing the lack of dynamic, repeatable assessment tools to evaluate and monitor EN skin disease severity, we conducted a structured exercise to assess agreement among experts on EN morphologic traits, sites of involvement, and the need for a skin severity assessment tool.”
The study also advised that extracutaneous manifestations might occur with the condition, affecting ocular, oropharyngeal, respiratory and/or genital membranes. In addition, psychological issues and chronic sequelae have been identified.
The researchers wrote that, not only do scoring systems for Stevens-Johnson syndrome and EN only estimate patient prognosis and are weighted toward comorbidities and systemic features, but morphologic terminology for EN lesions is inconsistent.
The study team sought to establish consensus among expert dermatologists on EN terminology, morphologic progression and most-affected sites. Another goal was to build a framework for developing a skin-directed scoring system for EN.
A core group from the Society of Dermatology Hospitalists initiated a Delphi consensus using the RAND/UCLA appropriateness criteria to establish agreement on the optimal design for an EN cutaneous scoring instrument, terminology, morphologic traits, and sites of involvement.
In an initial round, the 54 participating dermatology hospitalists reached consensus on all 49 statements (30 appropriate, 3 inappropriate, 16 uncertain). In the second round, they agreed on another 15 statements (8 appropriate, 7 uncertain).
Consistent agreement was reached on:
- need for a skin-specific instrument;
- most-often affected skin sites (head and neck, chest, upper back, ocular mucosa, oral mucosa); and
- blanching erythema, dusky erythema, targetoid erythema, vesicles/bullae, desquamation and erosions comprise the morphologic traits of EN and can be consistently differentiated.
“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,” the authors wrote. “It also established a baseline consensus for a standardized EN instrument with consistent terminology.”
- Waters M, Dobry A, Le ST, Shinkai K, 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.Rheumatology (Oxford)
