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Updated: Dec 17 2021

Stevens-Johnson Syndrome

Images syndrome.jpg
  • Snapshot
    • A young boy is brought to the emergency room after visiting his primary care physician, who noted erythematous, desquamative lesions all over his body (well over 30% of body surface area) and ulcerations of the mucosal membranes of the mouth and eyes. He was recently treated with penicillin for an infection.
  • Introduction
    • Stevens-Johnson Ssyndrome (SJS) and toxic epidermal necrolysis (TEN) – two diseases on the same spectrum
      • SJS: < 10% of body surface area
      • TEN: > 30% of body surface area
      • SJS/TEN overlap: 10 - 30% of body surface area
    • Severe, febrile blistering disease of skin and mucous membranes
      • often caused by drugs (>>> infection)
        • e.g., penicillin, sulfonamides, phenytoin, carbamazepine, lamotrigine, NSAIDs
      • can be caused by infection
        • e.g., Mycoplasma pneumonia
    • Immunocompromised patients like those with HIV or SLE are more at risk for SJS
    • Can be fatal
    • Erythema multiforme (EM) is a distinct disease from SJS/TEN according to the current consensus definition
  • Presentation
    • Symptoms
      • very painful skin
        • vs in EM, burning/pain is typically very mild
      • systemic signs
        • fever
        • dehydration
        • hypotension
      • non-skin findings
        • ocular involvement (50-80%)
          • corneal ulceration
          • uveitis
        • pulmonary involvement (25%)
          • pneumonitis
          • bronchiolitis obliterans
          • bronchitis
    • Physical exam
      • initially dusky red macules or patches (not raised)that progress to tense bullae and eventual skin sloughing
        • vs in EM, where lesions are typically papular/raised
      • mucous membranes always involved
        • bullae and erosions in oral, genital, anal mucosa
      • + Nikolsky sign (rubbing of skin easily causes sloughing – splitting of epidermis from dermis)
    • Most common cause of death is sepsis
  • Evaluation
    • Based on clinical history and symptoms
    • Skin biopsy: mainly to distinguish staphylococcal scalded skin syndrome and TEN
      • full-thickness epidermal necrosis
    • Labs: normal
  • Differential
    • Staphylococcal scalded skin syndrome
    • Graft versus host disease
    • Pemphigus vulgaris
    • Erythema multiforme
  • Treatment
    • Discontinue causative agent
      • early withdrawal = lower mortality
    • Hospitalization
      • admit to ICU or burn unit
      • if pulmonary involvement, may require mechanical ventilation
      • if ocular involvement, consult ophthalmology and apply topical erythromycin to prevent ocular adhesions
    • Supportive care
      • wound care with petrolatum and gauze
      • fluids, electrolytes, nutrition
    • Treat underlying infection
    • Pharmaceutical options to lower mortality: cyclosporine, IVIG, corticosteroids
      • all controversial
  • Complications
    • Skin hypo/hyperpigmentation
    • Ocular complications - dry eyes, corneal scarring, photophobia
    • Pulmonary complications - chronic bronchitis, bronchiectasis, bronchiolitis obliterans
    • Sepsis
  • Prognosis
    • High mortality
      • TEN: 30 - 50% mortality rate
      • SJS: 5 - 10% mortality rate
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