raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:300512

Onychomatricoma

Type
Disease
Group
Disorder
Inheritance
Unknown
Age of onset
All ages
Status
active

Onychomatricoma is a rare, benign nail tumor originating in the nail matrix characterized by localized or diffuse thickening of the nail plate, increased transverse or longitudinal overcurvature, a yellow longitudinal band of variable width, swelling of the proximal nail fold, multiple splinter hemorrhages and the presence of honeycomb-like cavities in the distal margin of the nail plate. Nail dystrophy and dorsal pterygium may be associated. Occasionally, a pigmented lesion has been reported.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enOnychomatricoma
frOnychomatricome
deOnychomatrikom
esOnicomatricoma
itOnicomatricoma
nlOnychomatricoom
ptOnicomatricoma
plGuz macierzy paznokcia
csOnychomatrikom
ukОніхоматрикома
zh甲膜瘤
trOnikomatrikom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D23.9NTBTattributedvalidated
MONDO0017587Evalidated
UMLSC4751078Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families50 casesWorldwideCase(s)Validated21965578[PMID]
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card