Toxic epidermal necrolysis
Synonyms: Lyell syndrome
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Toxic epidermal necrolysis | Lyell syndrome |
| fr | Nécrolyse épidermique toxique | NET · Syndrome de Lyell |
| de | Toxische epidermale Nekrolyse | Lyell-Syndrom |
| es | Necrólisis epidérmica tóxica | Síndrome de Lyell |
| it | Sindrome di Lyell | |
| nl | Toxische epidermale necrolyse | Syndroom van Lyell |
| pt | Necrólise epidérmica tóxica | Síndrome Lyell |
| pl | Zespół Lyella | Toksyczna nekroliza naskórka |
| cs | Lyellův syndrom | = en |
| uk | Токсичний епідермальний некроліз | Синдром Лайєлла |
| zh | 中毒性表皮坏死松解症 | Lyell综合征 |
| tr | Toksik epidermal nekroliz | Lyell sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L51.2 | NTBT | inclusion | validated |
| ICD-11 | EB13.1 | E | specific | validated |
| MONDO | 0018890 | E | validated | |
| UMLS | C0014518 | E | validated | |
| MedDRA | 10044223 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:95455Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum
rare skin diseases; rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
39 annotations · validated 2018-01-29| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0100792 | Acantholysis | |
| HP:0001875 | Decreased total neutrophil count | |
| HP:0002015 | Dysphagia | |
| HP:0010783 | Erythema | |
| HP:0012378 | Fatigue | |
| HP:0012733 | Macule | |
| HP:0001959 | Polydipsia | |
| HP:0100806 | Sepsis | |
| HP:0001873 | Thrombocytopenia | |
| HP:0001824 | Weight loss | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001903 | Anemia | |
| HP:0012735 | Cough | |
| HP:0100518 | Dysuria | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0003781 | Excessive salivation | |
| HP:0002024 | Malabsorption | |
| HP:0002205 | Recurrent respiratory infections | |
| Occasional (29-5%) | ||
| HP:0001637 | Abnormal myocardium morphology | |
| HP:0002103 | Abnormality of the pleura | |
| HP:0000795 | Abnormality of the urethra | |
| HP:0000142 | Abnormality of the vagina | |
| HP:0006554 | Acute hepatic failure | |
| HP:0000509 | Conjunctivitis | |
| HP:0200020 | Corneal erosion | |
| HP:0000621 | Entropion | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0031368 | Intestinal perforation | |
| HP:0002017 | Nausea and vomiting | |
| HP:0001733 | Pancreatitis | |
| HP:0000613 | Photophobia | |
| HP:0000083 | Renal insufficiency | |
| HP:0002098 | Respiratory distress | |
| HP:0002091 | Restrictive ventilatory defect | |
| HP:0200042 | Skin ulcer | |
| HP:0001645 | Sudden cardiac death | |
| HP:0002575 | Tracheoesophageal fistula | |
| HP:0000572 | Visual loss | |
Run the differential on the 11 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/537
- This record as JSON: /data/d/537.json