Stevens-Johnson syndrome and toxic epidermal necrolysis

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Background

  • SJS and TEN exist on a spectrum of disease
    • SJS involves <10% of BSA
    • TEN involves >30% of BSA
  • Dermatologic emergency
  • Causes:
    • Drugs - many. Common offensive agents include: quinolones, sulfa, PCN, ASA, acetaminophen, carbamazepine, NSAIDs, phenytoin, corticosteroids, immunizations
    • Malignancy - lymphoma
    • Idiopathic
    • Infectious

Clinical Features

  • Often have prodrome (fever, URI symptoms, HA, malaise)
  • Macular rash
    • +/- Target lesions
    • Usually starts centrally, spreads peripherally, and may become confluent
    • May be painful
    • May have +Nikolsky sign (denude when touched)
  • 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
  • Examine eyes/mucosal surfaces

DDx

  • Erythema Multiforme
  • Staphlococcal scalded skin syndrome
  • Erythroderma
  • Toxic Shock Syndrome
  • Drug eruption

Treatment

  • Removal of inciting cause if identified
  • Fluid replacement - treat shock w/ IV fluids according to burn protocols
  • Infection control
  • Wound care
  • Use of IVIG, plasmapheresis, and corticosteroids are controversial but may be beneficial

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 Rosen's UpToDate

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