Stevens - Johnson syndrome (SJS)

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:

• 1. Hackle J.G et al. Photo quiz - Rash in an adolescent. AFP 2004;70(3)
• 2. Gupta S, Devkaran A. Toxic epidermal necrolysis. Student BMJ 2008;16:168-170
• 3. Levi N et al. Medications as Risk Factors of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children: A Pooled Analysis. Pediatrics 2009;123: e297-e304 7
• 4. Segal A.R et al. Cutaneous Reactions to Drugs in Children. Pediatrics 2007;120:e1082-e1096
• 5. Lee EY, Knox C, Phillips EJ. Worldwide Prevalence of Antibiotic-Associated Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis. JAMA Dermatol. Published online February 15, 2023. doi:10.1001/jamadermatol.2022.6378
• 6. Jacobsen A, Olabi B, Langley A, Beecker J, Mutter E, Shelley A, Worley B, Ramsay T, Saavedra A, Parker R, Stewart F, Pardo Pardo J. Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome. Cochrane Database of Systematic Reviews 2022, Issue 3. Art. No.: CD013130. DOI: 10.1002/14651858.CD013130.pub2