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central areolar choroidal dystrophy

Pathology: - affect the posterior pole of the eye - early lesions consist of a nonspecific area of granular hyperpigmentation at the fovea - characteristic sign is a zone of atrophy that develops in the macula & involves the retinal pigment epithelium & the choriocapillaris, occurs several decades after onset Genetics: - autosomal dominant - associated with defects in PRPH2 (peripherin-2)

General

retinal disease genetic disease of the eye

Database Correlations

OMIM 215500

References

OMIM :accession 215500