What is the prognosis for an adult patient with Stevens-Johnson syndrome (SJS), potentially triggered by medication use or infection?

Stevens-Johnson Syndrome Prognosis

Stevens-Johnson Syndrome (SJS) carries a mortality rate of less than 10% when promptly recognized and managed, but survivors frequently face severe long-term complications that significantly impair quality of life. 1

Mortality Rates by Disease Severity

The prognosis varies substantially based on the extent of epidermal detachment:

• SJS alone (<10% body surface area): Mortality rate of 1-10% 1, 2
• SJS-TEN overlap (10-30% detachment): Mortality rate of approximately 14-15% 1
• TEN (>30% detachment): Mortality rate of 25-35% 2, 3

Children have dramatically better survival rates than adults, with pediatric SJS mortality near 0% compared to 3.1% in adults 1. This age-related difference is one of the strongest prognostic factors.

SCORTEN: Predicting Individual Risk

Calculate SCORTEN within the first 24 hours of admission to stratify mortality risk. 4, 1 This validated scoring system uses seven parameters:

• Age >40 years
• Presence of malignancy
• 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

Each parameter scores 1 point. Predicted mortality increases exponentially: 0-1 points = 1-4% mortality, 3 points = 32%, 5 points = 85%, and 7 points = 99% mortality 4.

Critical Prognostic Factors

Delayed transfer to a specialized center significantly increases mortality risk. 1 Other factors associated with worse outcomes include:

• Greater total body surface area involvement 1
• Development of septicemia (the most common cause of death) 4, 1
• Multiorgan failure 1
• Early pulmonary complications with bronchial epithelial necrolysis - carries 70% mortality when mechanical ventilation is required 4, 1

Acute Phase Complications

Pulmonary involvement is an underappreciated but critical marker of disease severity. 4 Three patterns exist:

• Group 1 (no hypoxemia): Excellent prognosis with low mortality 4
• Group 2 (early pulmonary manifestations): Bronchial epithelial necrolysis with 70% mortality, requiring mechanical ventilation in 90% of cases 4, 1
• Group 3 (delayed complications): Heterogeneous conditions including bacterial pneumonia and fluid overload 4

Cutaneous infection, particularly with Pseudomonas aeruginosa, impairs re-epithelialization and can lead to fatal sepsis. 4 Monitor carefully for signs beyond fever, including confusion, hypotension, reduced urine output, and oxygen desaturation 4.

Long-Term Sequelae and Quality of Life Impact

More than 50% of TEN survivors experience significant long-term morbidity that severely impacts quality of life. 2, 3 Common chronic complications include:

• Ocular sequelae: Chronic keratopathy, subconjunctival fibrosis, and vision impairment 4, 1
• Urogenital complications: Urethral strictures, phimosis in males, vaginal synechiae in females, causing urinary and sexual dysfunction 4, 1
• Respiratory problems: Bronchiolitis obliterans (particularly severe in children), bronchiectasis, chronic bronchitis with approximately 40% mortality 4, 1
• Cutaneous changes: Long-term pigmentary changes and nail dystrophy 4
• Psychological sequelae: Post-traumatic stress disorder is well-recognized 4, 1

Time Course and Recovery

Lesions reach maximum extent 5-7 days after disease onset. 1 Re-epithelialization may occur within days or take several weeks 4. Bronchial mucosa recovery parallels skin recovery in survivors. 1

Impact of Specialized Care

Rapid admission to a burns center or specialized intensive care unit with multidisciplinary team management is associated with improved survival. 1 High-quality supportive care remains the cornerstone of management, as there is no conclusive evidence demonstrating benefit of any specific immunomodulatory intervention over conservative management 4.

Common Pitfalls

Do not rely on fever alone to detect sepsis - the disease process itself causes fever, requiring vigilance for other signs of infection 4. Avoid indiscriminate prophylactic antibiotics, which may increase colonization with Candida albicans 4. Do not underestimate respiratory complications, which can occur at any stage including after discharge and require expert monitoring 4.