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 bsed on above total:

Score 0-1 (3.2%)

2  (12.1%)

3  (35.3%)

4  (58.3%)

5+  (90.0%)

Source

Tintinalli