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Original Investigation
March 6, 2019

Development and Validation of a Risk Prediction Model for In-Hospital Mortality Among Patients With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis—ABCD-10

Author Affiliations
  • 1Department of Dermatology, University of Pennsylvania, Philadelphia
  • 2Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania, Philadelphia
  • 3Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
  • 4Department of Dermatology, Duke University, Durham, North Carolina
  • 5Department of Dermatology, Stanford Hospital and Clinics, Redwood City, California
  • 6Science 37, Los Angeles, California
  • 7Department of Dermatology, Mayo Clinic, Rochester, Minnesota
  • 8Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas
  • 9Department of Dermatology, University of California San Francisco
  • 10Department of Dermatology, University of University of Alabama at Birmingham, Birmingham
  • 11Division of Dermatology, The Ohio State University Wexner Medical Center, Columbus
  • 12Department of Dermatology, Massachusetts General Hospital, Boston
  • 13Department of Dermatology, Stanford Hospital and Clinics, Stanford, California
  • 14Department of Dermatology, University of Minnesota, Rochester
  • 15Division of Dermatology, Washington University School of Medicine, St Louis, Missouri
  • 16Department of Dermatology, Oregon Health and Science University, Portland
  • 17Department of Dermatology, Hofstra Northwell School of Medicine, New Hyde Park, New York
  • 18Department of Dermatology, University of Utah School of Medicine, Salt Lake City
  • 19Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City
  • 20Division of Dermatology, University of California, Los Angeles
JAMA Dermatol. 2019;155(4):448-454. doi:10.1001/jamadermatol.2018.5605
Key Points

Question  Can a novel prognostic model for risk of in-hospital mortality be developed for Stevens-Johnson syndrome/toxic epidermal necrolysis in a contemporary, multi-institutional cohort of patients from the United States?

Findings  A 5-item mortality prediction model (ABCD-10; age, bicarbonate level, cancer, dialysis, and 10% involved body surface area) was developed from 370 patients. The model achieved good discrimination and calibration for predicting in-hospital mortality, although its performance was not significantly different from that of the SCORTEN model (Score of Toxic Epidermal Necrolysis).

Meaning  Among patients with Stevens-Johnson syndrome/toxic epidermal necrolysis, ABCD-10 accurately predicted in-hospital mortality; however, ABCD-10 requires validation in other cohorts to determine its generalizability.


Importance  Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a spectrum of severe mucocutaneous drug reaction associated with significant morbidity and mortality. A previously developed SJS/TEN-specific severity-of-illness model (Score of Toxic Epidermal Necrolysis [SCORTEN]) has been reported to overestimate and underestimate SJS/TEN-related in-hospital mortality in various populations.

Objective  To derive a risk prediction model for in-hospital mortality among patients with SJS/TEN and to compare prognostic accuracy with the SCORTEN model in a multi-institutional cohort of patients in the United States.

Design, Setting, and Participants  Data from a multicenter cohort of patients 18 years and older treated for SJS/TEN between January 1, 2000, and June 1, 2015, were obtained from inpatient consult databases and electronic medical record systems at 18 medical centers in the United States as part of the Society for Dermatology Hospitalists. A risk model was derived based on data from 370 of these patients. Model discrimination (calculated as area under the receiver operating characteristic curve [AUC]) and calibration (calculated as predicted vs observed mortality, and examined using the Hosmer-Lemeshow goodness-of-fit statistic) were assessed, and the predictive accuracy was compared with that of SCORTEN. All analysis took place between December 2016 and April 2018.

Main Outcomes and Measures  In-hospital mortality.

Results  Among 370 patients (mean [SD] age 49.0 [19.1] years; 195 [52.7%] women), 54 (15.14%) did not survive to hospital discharge. Five covariates, measured at the time of admission, were independent predictors of in-hospital mortality: age in years (odds ratio [OR], 1.05; 95% CI, 1.02-1.07), body surface area (BSA) in percentage of epidermal detachment (OR, 1.02; 95% CI, 1.01-1.04), serum bicarbonate level below 20 mmol/L (OR, 2.90; 95% CI, 1.43-5.88), active cancer (OR, 4.40; 95% CI, 1.82-10.61), and dialysis prior to admission (OR, 15.94; 95% CI, 3.38-66.30). A severity-of-illness score was calculated by taking the sum of 1 point each for age 50 years or older, epidermal detachment greater than 10% of BSA, and serum bicarbonate level below 20 mmol/L; 2 points for the presence of active cancer; and 3 points for dialysis prior to admission. The score was named ABCD-10 (age, bicarbonate, cancer, dialysis, 10% BSA). The ABCD-10 model showed good discrimination (AUC, 0.816; 95% CI, 0.759-0.872) and calibration (Hosmer-Lemeshow goodness of fit test, P = .30). For SCORTEN, on admission, the AUC was 0.827 (95% CI, 0.774-0.879) and was not significantly different from that of the ABCD-10 model (P = .72).

Conclusions and Relevance  In this cohort of patients with SJS/TEN, ABCD-10 accurately predicted in-hospital mortality, with discrimination that was not significantly different from SCORTEN. Additional research is needed to validate ABCD-10 in other populations. Future use of a new mortality prediction model may provide improved prognostic information for contemporary patients, including those enrolled in observational studies and therapeutic trials.