Orphanet: Central areolar choroidal dystrophy

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Central areolar choroidal dystrophy

Disease definition

A hereditary macular disorder, usually presenting between the ages of 30-60, characterized by a large area of atrophy in the centre of the macula and the loss or absence of photoreceptors, retinal pigment epithelium and choriocapillaris in this area, resulting in a progressive decrease in visual acuity.


Classification level: Disorder
  • Synonym(s):
    • Areolar atrophy of the macula
    • CACD
    • Central areolar choroidal sclerosis
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: H31.2
  • ICD-11: 9B61
  • OMIM: 215500  613105  613144
  • UMLS: C1536451
  • MeSH: C535358
  • GARD: 10049
  • MedDRA: -

Detailed information

General public


ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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