Stevens–johnson Syndrome

Basics

Description

  • Stevens–Johnson syndrome (SJS) is a disease that affects the skin and mucous membranes, causing necrosis and detachment of the epidermal layers
  • SJS is either drug induced, related to infection, or idiopathic
  • SJS and toxic epidermal necrolysis (TEN) are considered a disease continuum and differ only in the amount of body surface involvement:
    • SJS: <10% of body surface area (BSA)
    • SJS–TEN overlap syndrome: 10–30% of BSA
    • TEN: >30% of BSA, can affect up to 100% BSA
    • SJS and TEN are different from erythema multiforme
  • SJS characteristics:
    • Blistering of <10% of the BSA
    • 95% of patients have mucous membrane lesions:
      • Usually at 2 or more sites
      • Ocular, oral, genital
    • 85% have conjunctival lesions
    • Lesions often involving face, neck, and central trunk regions become confluent over hours to days
  • Age of onset is any age, but most common in adults older than 40
  • More common in women, female to male ratio of 2:1

Etiology

  • The most common causes include medications and infections:
    • The pathology of skin damage is not completely understood
    • Damage to the skin is thought to be mediated by a cell mediated cytotoxic immune reaction aimed at destruction of keratinocytes expressing (drug-related) antigens
      • Leads to massive apoptosis
    • Medications cause the vast majority of SJS, usually in the first 8 wk of treatment
  • Causative medications:
    • Antibiotics (penicillin, sulfonamides, others)
    • Anticonvulsants (phenytoin, phenobarbital, carbamazepine, lamotrigine, others)
    • NSAIDs
    • Antineoplastics (thalidomide, tamoxifen, others)
    • HIV drugs (nevirapine)
    • Allopurinol
  • Infections:
    • Mycoplasma pneumoniae is the second most common cause of SJS
    • Herpes simplex
    • Viral infections

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