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

Stevens–Johnson syndrome

Mucosal lesions with Stevens-Johnson

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

Differential Diagnosis

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

Diagnosis

Work-Up

• CBC
• CMP
• ESR
• CXR
• Examine eyes/mucosal surfaces

Evaluation

• Clinical diagnosis

Management

• 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

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%)

Disposition

• Admit to burn unit or ICU

See Also

• Rashes