Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Toxic epidermal necrolysis
Synonyms

Lyell syndrome

Definitions

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.

ID

http://www.orpha.net/ORDO/Orphanet_537

alternative_term

Lyell syndrome

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.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=537

has_age_of_onset

All ages

has_inheritance

Not applicable

hasDbXref

UMLS:C0014518

ICD-11:EB13.1

ICD-10:L51.2

MedDRA:10044223

label

Toxic epidermal necrolysis

notation

Clinical subtype

ORPHA:537

part_of

http://www.orpha.net/ORDO/Orphanet_95455

prefixIRI

ORDO:Orphanet_537

prefLabel

Toxic epidermal necrolysis

present_in

Worldwide AND has_point_prevalence_range : Unknown

treeView

http://www.orpha.net/ORDO/Orphanet_95455

subClassOf

http://www.orpha.net/ORDO/Orphanet_557494

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