Zespół Stevensa i Johnsona

Kod Orpha: 36426Kod OMIM: 608579

Definicja

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.

Dane
Klasyfikacja

Podtyp kliniczny

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

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