Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome
Synonyms: SJS/TEN overlap syndrome · Stevens-Johnson/TEN overlap syndrome · Stevens-Johnson/toxic epidermal necrolysis overlap syndrome
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome | SJS/TEN overlap syndrome · Stevens-Johnson/TEN overlap syndrome · Stevens-Johnson/toxic epidermal necrolysis overlap syndrome |
| fr | Syndrome de chevauchement du syndrome de Stevens-Johnson/nécrolyse épidermique toxique | Syndrome de chevauchement SJS/NET |
| de | SJS/TEN-Übergangsform | SJS mit Übergang in toxisch-epidermale Nekrolyse · SJS/TEN-Overlap-Syndrom · Stevens-Johnson/toxische epidermale Nekrolyse-Überlappungs-Syndrom · Übergangsform Stevens-Johnson-Syndrom/toxische epidermale Nekrolyse |
| es | Síndrome de solapamiento SJS/TEN | Síndrome de solapamiento Stevens-Johnson/TEN · Síndrome de solapamiento Stevens-Johnson/necrólisis epidérmica tóxica · Síndrome de solapamiento del síndrome de Stevens-Johnson/necrólisis epidérmica tóxica |
| it | Sindrome sovrapposta a SJS/TEN | |
| nl | SJS/TEN-overlapsyndroom | Stevens-Johnson/TEN-overlapsyndroom · Stevens-Johnson/toxische epidermale necrolyse-overlapsyndroom · Syndroom van Stevens-Johnson/toxische epidermale necrolyse-overlapsyndroom |
| pt | Síndrome de sobreposição a síndrome Stevens-Johnson/necrólise epidérmica tóxica | |
| pl | Zespół Stevensa i Johnsona/zespół z nakładającymi się objawami typu toksycznej nekrolizy naskórka | = en |
| cs | Přesahující syndrom SJS/TEN | Přesahující syndrom Stevensův-Johnsonův/TEN · Přesahující syndrom Stevensův-Johnsonův/toxické epidermální nekrolýzy · Stevensův-Johnsonův syndrom/Syndrom přesahující toxické epidermální nekrolýzy · Přesahující syndrom Stevens-Johnsonův/TEN · Přesahující syndrom Stevens-Johnsonův/toxické epidermální nekrolýzy · Stevens-Johnsonův syndrom/Syndrom přesahující toxické epidermální nekrolýzy |
| uk | Синдром Стівенса-Джонсона/Overlap-синдром токсичного епідермального некролізу | Синдром перекриття SJS/TEN · Синдром перекриття Стівенса-Джонсона/TEN · Синдром Стівенса-Джонсона/ накладання токсичного епідермального некролізу |
| zh | Stevens-Johnson综合征/毒性表皮坏死松解综合征 | Stevens-Johnson/毒性表皮坏死松解综合征 |
| tr | Stevens-Johnson sendromu/toksik epidermal nekroliz ile örtüşen sendrom | Stevens-Johnson/toksik epidermal nekroliz örtüşen sendrom · SJS/TEN örtüşen sendrom · Stevens-Johnson / TEN örtüşen sendrom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L51.2 | NTBT | attributed | validated |
| ICD-11 | EB13.2 | E | specific | validated |
| MONDO | 0044739 | E | validated | |
| UMLS | C3661878 | 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
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/506784
- This record as JSON: /data/d/506784.json