A Retrospective Cohort Study of the Management and Outcomes of Children Hospitalized with Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis

Abstract

Background: Severe cutaneous adverse reactions are rare yet life-threatening conditions. The current management and outcomes of these conditions in US children are unclear.

Objective: To characterize the current management and outcomes of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) across US children's hospitals.

Methods: We performed a retrospective cohort study of children younger than 18 years hospitalized with a primary diagnosis of SJS or TEN at 47 US freestanding children's hospitals. We compared treatment (intravenous immunoglobulin [IVIG], steroids, antibiotics, and others) and outcomes (length of stay [LOS], hospital mortality, readmission, recurrence, related complications, and adjusted hospital costs) across hospitals and by SJS versus TEN diagnoses.

Results: We identified 898 pediatric patients hospitalized with a primary diagnosis of SJS or TEN. Of these patients, 167 (18.6%) were prescribed steroids only, 229 (25.5%) IVIG only, and 153 (17.04%) both IVIG and steroids. Median LOS was 8 days (interquartile range, 5-13) with median hospital-adjusted costs of $16,265. Readmissions were common, with 88 (9.9%) patients readmitted within 30 days of discharge and a recurrence rate of 2.7%. Overall hospital mortality in children was low at 0.56%. TEN was associated with higher mortality (3.23%) compared with SJS (0.13%). There was no association between the use of IVIG, systemic steroids, or IVIG and steroids during the first 2 days of hospitalization and decreased LOS or mechanical ventilation. Complex chronic conditions and TEN diagnoses were associated with increased LOS and increased odds of mechanical ventilation.

Conclusion: Survival in children with SJS and TEN is significantly better than that observed in adults. However, there is variability in the management and outcomes in children diagnosed with these severe cutaneous reactions. Further studies are needed to determine the most effective treatment strategies given the extent of health care utilization and high rate of readmissions observed in this population.

Keywords: Drug adverse effects; Pediatric hospital medicine; Severe cutaneous adverse reactions; Stevens-Johnson syndrome; Toxic epidermal necrolysis.