Nevus of Ota
Synonyms: Nevus fusculoceruleus ophthalmomaxillaris
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Nevus of Ota is an oculodermal melanocytosis more commonly found in Asian and African populations, usually present at birth and characterized by a usually unilateral, bluish gray, patchy, speckled pigmentation (that may progressively enlarge and darken) affecting the skin of the face along the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve (periorbital region, temple, forehead, malar area, nose). In 2/3 cases the ipsilateral sclera is affected. Nevus of Ota usually remains stable once adulthood is reached but an increased risk of glaucoma and uveal melanoma may be observed. Extracutaneous lesions may also occur in cornea, retina, tympanum, nasal mucosa, pharynx, palate. Nevus of Ota occurs as solitary conditions but seldom may occur together with the nevus of Ito or nevus spilus.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Nevus of Ota | Nevus fusculoceruleus ophthalmomaxillaris |
| fr | Naevus d'Ota | |
| de | Naevus Ota | |
| es | Nevo de Ota | Nevo fusco-cerúleo oftálmico maxilar |
| it | Nevo di Ota | Nevus fusculocoeruleus ophthalmomaxillaris |
| nl | Naevus van Ota | Naevus fusculocoeruleus ophthalmomaxillaris |
| pt | Nevus de Ota | |
| pl | Znamię Oty | Znamię okulodermalne |
| cs | Otův névus | = en |
| uk | Невус Ота | Фускулоцеребральний невус офтальмомаксилярний |
| zh | 太田痣 | 眼上腭部褐青色痣 |
| tr | Ota nevüsü | Nevüs fussuloserulös oftalmo maksillaris |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D22.3 | NTBT | attributed | validated |
| ICD-11 | LC10 | NTBT | index | validated |
| MONDO | 0016984 | E | validated | |
| MeSH | D009507 | E | validated | |
| UMLS | C0027961 | E | validated | |
| MedDRA | 10051713 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/263425
- This record as JSON: /data/d/263425.json