Stevens-Johnson syndrome

Orpha code: 36426OMIM code: 608579

Definition

A limited form of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum characterized by destruction and detachment of the skin epithelium, involving less than 10% of the body surface area, and mucous membranes. Onset usually occurs 4-28 days after administration of the causal medication and is most frequently associated with anticonvulsants, antibacterial sulfonamides, allopurinol, nevirapine, and oxicams (non-steroidal anti-inflammatory drugs), but many other medications have also been implicated. The disease is not induced by medication in 15% of cases. Histology is characterized by an epidermal necrolysis. Multiple disabling long-term sequelae (especially cutaneous, ocular and psychological) are frequent.

Disease data
Classification

Clinical subtype

Synonyms
Dermatostomatitis, Stevens Johnson type
Dermatostomatitis, Stevens Johnson type
ORPHA code
36426
OMIM code
608579
ICD10 code
L51.1
ICD11 code
EB13.0

No additional description.

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