Stevens-Johnson syndrome

Background

• A severe cutaneous adverse reaction

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

Clinical Manifestations

• Onset typically 1 to 3 weeks, but ranges from a few days up to 8 weeks
• Characterized by large blisters, large areas of skin detachment, confluent areas of erythema, atypical target lesions, purpura
• Nikolsky sign
• Classified as SJS if less than 10% body surface area involved, or TEN if ≥30%
• May have fever or influenza-like illness
• May have respiratory symptoms
• May have lymphopenia and AKI

Prognosis

• Mortality is 10 to 40%, depending on severity
• Can develop superimposed bacterial infections
• After withdrawal of the offending drug, re-epithelialization starts around 1 week and lasts up to 3 weeks
• Can result in permanent scarring, hyperpigmentation, alopecia, nail loss, vision loss, synechiae, dry eye, dental problems

Differential Diagnosis

• Erythema multiforme
• Coxsackievirus
• Mycoplasma pneumoniae
• Linear IgA bullous dermatosis
• Generalized bullous fixed-drug eruption
• Methotrexate toxicity
• Graft-versus-hosts disease
• Staphylococcal scalded skin syndrome
• Systemic lupus erythematosus
• Pemphigus vulgaris

Diagnosis

• Diagnosis is based on histopathology of a skin biopsy, which will show full-thickness epidermal necrosis, focal adnexal necrosis, necrotic keratinocytes, and mild mononuclear cell dermal infiltrate
  • Negative direct immunofluorescence tests

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 30º to 32ºC
  • 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