raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:228272

Primary anetoderma

Synonyms: Primary macular atrophy

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
All ages
Status
active

Primary anetoderma is a rare skin disease characterized by loss of elastin tissue resulting in localized areas of flaccid skin in the absence of a secondary cause.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPrimary anetodermaPrimary macular atrophy
frAnétodermie primitiveAtrophie maculaire primitive
deAnetodermie, primäreMakulaatrophie, primäre
esAnetodermia primariaAtrofia macular primaria
itAnetoderma primitivoAtrofia maculare primitiva
nlPrimaire anetodermiePrimaire macula-atrofie
ptAnetoderma primário
plPierwotny zanik skórny plackowatyPierwotny zanik plamki
csPrimární anetodermiePrimární makulární atrofie
ukПервинна анетодерміяПервинна макулярна атрофія
zh原发性皮肤松垂原发性斑状萎缩
trPrimer anetodermaPrimer maküler atrofi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10L90.1BTNTspecificvalidated
ICD-10L90.2BTNTspecificvalidated
ICD-11EE41.1NTBTindexvalidated
MONDO0016444Evalidated
MeSHD057088Evalidated
UMLSC0406550Evalidated

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

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

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