Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome

Orpha code: 506784OMIM code:

Definition

An intermediate form of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum characterized by destruction and detachment of the skin epithelium, involving between 10 to 29% 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
SJS/TEN overlap syndrome
Stevens-Johnson/TEN overlap syndrome
Stevens-Johnson/toxic epidermal necrolysis overlap syndrome
SJS/TEN overlap syndrome
Stevens-Johnson/TEN overlap syndrome
Stevens-Johnson/toxic epidermal necrolysis overlap syndrome
ORPHA code
506784
OMIM code
-
ICD10 code
L51.2
ICD11 code
-

No additional description.

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