Stevens-Johnson syndrome and toxic epidermal necrolysis
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
Differential Diagnosis
- 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