Stevens - Johnson syndrome (SJS)

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Stevens Johnson syndrome is a severe and sometimes fatal form of erythema multiforme

  • there is more widespread skin and mucosal involvement than in erythema multiforme (1)

  • although erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) were thought to belong to the same group of disorders there is supporting evidence that EM differs from the other 2 diseases in:
    • clinical features
    • severity
    • cause
    • dermographic variables (2)

  • SJS is a rare, life threatening mucocutaneous condition with epidermal detachment, acute skin blisters and erosions in the mucous membranes (3)

  • detachment is seen in less than 10% of the total body surface areas (3)

  • the disease can be seen in all ages including children, infants and sometimes in newborns as well (2)

  • there is a higher incidence in children and young adults, and it is twice as common in males than females. There may be pulmonary, gastrointestinal, cardiac or renal involvement (4)

With respect to systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome (5):

  • found when compared to corticosteroids, etanercept may result in mortality reduction
  • certainty of evidence for disease-specific mortality is very low for corticosteroids versus no corticosteroids, IVIG (intravenous immunoglobulins) versus no IVIG and cyclosporin versus IVIG

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Last edited 04/2022 and last reviewed 05/2022

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