Stevens - Johnson syndrome (SJS)

Last edited 02/2023 and last reviewed 10/2023

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)

  • study evidence showed that antibiotics were associated with more than one-quarter of SJS/TEN cases described worldwide, and sulfonamide antibiotics remained the most important association (5)

With respect to systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome (6):

  • 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

Reference:

Related pages:

aetiology

clinical features

differential diagnosis

management

prognosis