Prognosis of Stevens-Johnson Syndrome
Stevens-Johnson Syndrome carries a mortality rate of less than 10%, with overall survival exceeding 90% when promptly recognized and appropriately managed, though survivors frequently face significant long-term sequelae affecting quality of life. 1
Mortality Rates by Disease Severity
The prognosis varies significantly based on the extent of epidermal detachment:
- SJS (< 10% body surface area detachment): Mortality < 10% 1
- SJS-TEN overlap (10-30% detachment): Mortality approximately 14-15% 1
- TEN (> 30% detachment): Mortality rises to 25-35% 1, 2
- Overall SJS/TEN spectrum mortality: Approximately 22% 1
Pediatric vs. Adult Outcomes
Children and young people have substantially better survival rates than adults. 1
- Pediatric SJS mortality: 0% 1
- Pediatric SJS-TEN overlap mortality: 2.5-3.98% 1
- Pediatric TEN mortality: 4.2-14.73% 1
- Adult SJS mortality: 3.1% 1
- Adult TEN mortality: 17% 1
A critical caveat: Recurrence is more common in children (up to 18% of cases) because the precipitant is usually infection rather than drugs, which can recur despite avoidance measures. 1
Prognostic Scoring: SCORTEN
SCORTEN should be calculated within the first 24 hours of admission to predict mortality risk. 1
The SCORTEN system uses seven clinical parameters, with each parameter contributing one point:
- Age > 40 years
- Malignancy present
- Heart rate > 120 bpm
- Initial body surface area detachment > 10%
- Serum urea > 10 mmol/L
- Serum glucose > 14 mmol/L
- Serum bicarbonate < 20 mmol/L
Higher SCORTEN scores correlate with increased mortality rates, and the score typically rises slightly during hospitalization (significant difference between day 1 and day 4). 1
Factors Associated with Worse Prognosis
Several factors predict increased mortality and morbidity:
- Delayed transfer to specialist unit (associated with increased mortality) 1
- Increasing patient age 1
- Greater total body surface area involvement 1
- Presence of septicemia (the most frequent cause of death) 1, 3
- Granulocytopenia 1
- Multiorgan failure 1
Acute Phase Complications
During the acute illness, patients face multiple life-threatening complications:
- Septicemia (leading cause of morbidity and mortality) 3
- Multiorgan failure 1
- Pulmonary complications (marker of disease severity; 70% mortality in those with early bronchial epithelial necrolysis requiring mechanical ventilation) 1
- Thermoregulatory dysfunction and hemodynamic instability 1
- Renal impairment and liver dysfunction 1, 3
Long-Term Sequelae and Quality of Life
Even among survivors, significant long-term morbidity is common and severely impacts quality of life. 1
Common chronic complications include:
- Ocular sequelae (most common long-term complication) 1, 3
- Mucocutaneous complications 1, 3
- Psychological sequelae 1
- Urogenital strictures and dysfunction (urethral strictures, phimosis, vaginal synechiae) 1
- Chronic respiratory problems (bronchiolitis obliterans, bronchiectasis, chronic bronchitis with approximately 40% mortality) 1
- Nasopharyngeal, esophageal, and genital strictures 3
More than 50% of TEN survivors experience long-term reduced quality of life and decreased life expectancy. 4
Impact of Specialized Care on Outcomes
Rapid admission to a burns center or specialized intensive care unit is associated with improved survival. 1
Three studies and a systematic review demonstrate that:
- Early transfer to specialized centers improves survival 1
- Delayed transfer increases mortality 1
- Multidisciplinary team management coordinated by skin failure specialists reduces complications 1
Time Course of Recovery
For survivors, the acute phase typically follows this pattern: