Pellucid marginal degeneration
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare disorder of the anterior segment of the eye characterized by slowly progressive, bilateral, non-ulcerative, non-inflammatory, clear thinning of the inferior portion of the peripheral cornea (extending from the 4 o'clock to the 8 o'clock position), with an area of corneal protrusion above the point of maximal thinning, resulting in against-the-rule astigmatism with decreased visual acuity. The central cornea is of normal thickness.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pellucid marginal degeneration | |
| fr | Dégénérescence marginale pellucide | |
| de | Hornhautdegeneration, marginale pelluzide | |
| es | Degeneración marginal pelúcida | |
| it | Degenerazione marginale pellucida | |
| nl | Heldere marginale degeneratie | |
| pt | Degeneração marginal pelúcida | |
| pl | Zwyrodnienie brzeżne przezroczyste | |
| cs | Pelucidní marginální degenerace | |
| uk | Пелюцідна крайова дегенерація (рогівки) | |
| zh | 边缘角膜透明变性 | |
| tr | Pellusid marjinal dejenerasyon |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H18.7 | NTBT | attributed | validated |
| ICD-11 | 9A78.4 | NTBT | index | validated |
| MONDO | 0015298 | E | validated | |
| UMLS | C0339288 | E | validated | |
| GARD | 11895 | 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:519288Rare disorder with corneal involvement as a major feature
rare ophthalmic disorders
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/137672
- This record as JSON: /data/d/137672.json