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Stevens-Johnson syndrome

ORPHA36426
Synonym(s) Dermatostomatitis, Stevens Johnson type
Prevalence Unknown
Inheritance Not applicable
Age of onset All ages
ICD-10
  • L51.1
OMIM
UMLS
  • C0038325
MeSH
  • D013262
MedDRA
  • 10042033

Summary

Stevens-Johnson syndrome is a limited form of toxic epidermal necrolysis (see this term) characterized by destruction and detachment of the skin epithelium and mucous membranes involving less than 10% of the body surface area.

The annual incidence of Stevens-Johnson syndrome is less than 1/1,000,000.

Stevens-Johnson syndrome can be triggered by a drug allergy and, more rarely, by infections or bone marrow transplantation. In 25 to 30% of cases, the cause is unclear.

Patients should be admitted to an intensive care or burns unit as soon as the diagnosis is suspected.

Reepithelialization is rapid (2-3 weeks).

Expert reviewer(s)

  • Pr Jean-Claude ROUJEAU

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Detailed information

Emergency guidelines
  • FR (2012,pdf)
Review article
  • FR (2007,pdf)
  • EN (2010)
Clinical practice guidelines
  • FR (2010,pdf)
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