Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis

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Definition

  • Stevens-Johnson Syndrome (SJS): <10% of total body surface area affected
  • Toxic Epidermal Necrolysis (TEN): >30% of total body surface area affected
  • SJS/TEN: 10-30% of total body surface area affected

Aetiology

  • Medications (75%): including sulfonamides/sulfasalazine, allopurinol, antiepileptics (e.g., lamotrigine, phenobarbital), SSRIs, NSAIDs
  • Infections (especially mycoplasma)

Ocular Findings

  • Acute pseudomembranous conjunctivitis, subconjunctival haemorrhages, formation of membranes/pseudomembranes
  • Cornea: persistent epithelial defects, erosions, ulcers, vascularisation, scarring
  • Symblepharon (adhesion between bulbar and palpebral conjunctiva)
  • Fibrosed/scarred tear ducts, destruction of goblet cells (-> dry eye), entropium with trichiasis

Therapy in Acute Phase

  • Local steroid therapy
    • e.g., Ultracortenol ointment 4-6x/day or Dexafree UD 4x/day (depending on severity)
  • Local antibiotic therapy: e.g., Floxal UD 3x/day
  • Heparin Eye Drops 5000 IU/ml 4x/day to prevent membrane formation
  • Lubricating therapy: e.g., Lacrycon gtt 4x/day to hourly
  • Daily sweeping of the fornices with a glass spatula + Ultracortenol ointment until significant improvement +/- membrane removal
  • Daily rinsing for detritus removal
  • Apply preservative-free medications whenever possible!
  • Consider amniotic membrane transplant in severe cases

Sources

  • EyeWiki Stevens-Johnson Syndrome
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)