Stevens-Johnson syndrome and toxic epidermal necrolysis

Background

• SJS and TEN involve immune-complex mediated cell death w/ separation of epidermis from dermis
  • SJS involves <10% of BSA
  • TEN involves >30% of BSA

Diagnosis

• Often have prodrome (fever, URI symptoms, HA, malaise)
• Rash begins suddenly
  • Target lesions
• Mucous membranes can be severely affected
  • Eye involvement can be severe
• In severe cases, respiratory tract and GI involvement may occur

Work-Up

• CBC
• Chem
• ESR
• CXR

Treatment

• Removal of inciting cause if identified
• Treat shock w/ IV fluids according to burn protocols

Disposition

• Admit to burn unit or ICU

Prognosis

Validated with SCORTEN mortality assessment:

One point for each of the following assessed within 1st 24 hours of admission:

• Age >/= 40 years (OR 2.7)
• Heart Rate >/= 120 beats per minute (OR 2.7)
• Cancer/Hematologic malignancy (OR 4.4)
• Body surface area on day 1  >10% (OR2.9)
• Serum urea level (BUN) >28mg/dL (>10mmol/L) (OR 2.5)
• Serum bicarbonate <20mmol/L (OR 4.3)
• Serum glucose > 252mg/dL (>14mmol/L) (OR5.3)

Predicted mortality based on above total:

Score 0-1 (3.2%)

2  (12.1%)

3  (35.3%)

4  (58.3%)

5+  (90.0%)

Source

Tintinalli

Sylvie Bastuji-Garin, Nathalie Fouchard*, M Bertocchi*, Jean-Claude Roujeau*, Jean Revuz* and Pierre Wolkenstein. SCORTEN: A Severity-of-Illness Score for Toxic Epidermal Necrolysis. Journal of Investigative Dermatology (2000) 115, 149–153

Rob Cartotto, MD, FRCS(C), Mike Mayich, BSc, Duncan Nickerson, MD, FRCS(C), Manuel Gomez, MD, MSc. SCORTEN Accurately Predicts Mortality Among Toxic Epidermal Necrolysis Patients Treated in a Burn Center. J Burn Care Res 2008;29:141–146