Toxic epidermal necrolysis

Orpha code: 537OMIM code:

Definition

An extended form of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum characterized by destruction and detachment of the skin epithelium, involving 30% or more 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
Lyell syndrome
Toksyczna nekroliza naskórka
ORPHA code
537
OMIM code
-
ICD10 code
L51.2
ICD11 code
EB13.1

No additional description.

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