Updated: 12/13/2019

Stevens-Johnson Syndrome

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Snapshot
  • This clinical pictures shows extensive mucosal involvement in the blistering skin disease, SJS/TENA 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
Prognosis, Prevention, and Complications
  • Prognosis
    • high mortality
      • TEN: 30 - 50% mortality rate
      • SJS: 5 - 10% mortality rate
  • Complications
    • skin hypo/hyperpigmentation
    • ocular complications - dry eyes, corneal scarring, photophobia
    • pulmonary complications - chronic bronchitis, bronchiectasis, bronchiolitis obliterans
    • sepsis

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