NEVADA syndrome
Synonyms: Nevus epidermicus verrucosus with angiodysplasia and aneurysms
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare, life-threatening, cutaneous disease characterized by a keratinocytic epidermal nevus presenting thick, hystrix-like, white or brownish hyperkeratosis associated with multiple extracutaneous vascular malformations, including angiodysplasia that involves large-vessel arteriovenous shunts that may be fatal during the neonatal period.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | NEVADA syndrome | Nevus epidermicus verrucosus with angiodysplasia and aneurysms |
| fr | Syndrome NEVADA | Naevus épidermique verruqueux avec angiodysplasie et anévrismes |
| de | NEVADA-Syndrom | Naevus verrucosus mit Angiodysplasie und Aneurysmen |
| es | Síndrome NEVADA | Nevo epidérmico verrugoso con angiodisplasia y aneurismas |
| it | Sindrome NEVADA | Nevo epidermico verrucoso con angiodisplasia e aneurismi |
| nl | NEVADA-syndroom | Naevus epidermicus verrucosus met angiodysplasie en aneurysmata |
| pt | Sindrome NEVADA | |
| pl | Zespół NEVADA | Znamię naskórkowe brodawkowate z angiodysplazją i tętniakami |
| cs | NEVADA syndrom | Nevus epidermicus verrucosus s angiodysplázii a aneuryzmaty |
| uk | Синдром NEVADA | Веррукозний епідермальний невус з ангіодисплазією та аневризмами |
| zh | NEVADA综合征 | 表皮疣状痣伴血管发育不良和动脉瘤 |
| tr | NEVADA sendromu | Anjiyodisplazi ve anevrizmalarla seyreden Nevus epidermicus verrucosus |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q87.8 | NTBT | attributed | validated |
| ICD-11 | BE2Y | NTBT | attributed | validated |
| MONDO | 0018261 | E | validated | |
| UMLS | C4751431 | 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:294057Rare nevus
rare skin diseases; rare neoplastic diseases
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/370059
- This record as JSON: /data/d/370059.json