Onychocytic matricoma
Synonyms: Acanthoma of the nail matrix
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare, benign, nail tumor originating in the nail matrix characterized by localized pachyonychia and variable degrees of pigmentation: pigmented, melanocytic (common, longitudinal melanonychia that may simulate a foreign body) or hypopigmented. Histopathology demonstrates a purely epithelial tumor with endokeratinization in the deep portion and concentrically arranged nests of prekeratogenous and keratogenous cells.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Onychocytic matricoma | Acanthoma of the nail matrix |
| fr | Matricome onychocytique | Acanthome de la matrice des ongles · Acanthome de la matrice unguéale |
| de | Onychozytisches Matrikom | Akanthom der Nagelmatrix |
| es | Matricoma onicocítico | Acantoma de la matriz ungueal |
| it | Matricoma onicocitico | Acantoma della matrice ungueale |
| nl | Onychocytisch matricoom | Acanthoom van nagelmatrix |
| pt | Matricoma onicocítico | |
| pl | Rogowiak macierzy paznokcia | Rogowiak macierzy paznokcia · Akantoza macierzy paznokcia |
| cs | Onychocytární matrikom | Akantom matrix nehtů |
| uk | Оніхоцитарна матрикома | Акантома нігтьового матриксу |
| zh | 甲细胞间质瘤 | 甲基质棘皮瘤 |
| tr | Onikositik matrikom | Tırnak matrisinin akantoması |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D23.9 | NTBT | attributed | validated |
| MONDO | 0017586 | E | validated | |
| UMLS | C4751108 | E | validated | |
| GARD | 11007 | 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:300515Rare nail tumor
rare skin diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 5 cases | Worldwide | Case(s) | Validated | 22197859[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/300504
- This record as JSON: /data/d/300504.json