Stevens-Johnson syndrome

Etiology

• Infection: especially common in children
  • Mycoplasma pneumoniae: moderate to severe involvement of two or more mucosal sites and sparse, or even absent, skin involvement
  • Neisseria gonorrhoeae1
• Drugs
  • Allopurinol
  • Aromatic antiepileptic drugs and lamotrigine
  • Antibacterial sulfonamides (including sulfamethoxazole and sulfasalazine)
  • Nevirapine
  • Oxicam nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Less strongly, other antibiotics
    • Doxycycline
    • Amoxicillin/ampicillin
    • Ciprofloxacin
    • Levofloxacin
    • Rifampin

Management

• Risk stratify with SCORTEN score
• Consider referral to burn centre or ICU if BSA 30% or greater, or if SCORTEN score is 2 or greater
• Stop offending drug, if applicable
• Supportive care, including wound care and IV fluids (2 ml per kg times BSA over first 24 hours)
  • Room temperature 30 to 32 C
  • May need NG tube
  • Pain control, as pain may be severe
• Ophthalmologic exam and management
  • Saline rinses
  • Artificial tears even if no involvement
  • Corticosteroids with or without antibiotics for any involvement
• No clear benefit to steroids or IVIG
• Cyclosporine 3 to 5 mg per kg in one or two divided doses may slow progression