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Pigmented paravenous retinochoroidal atrophy

Synonym(s) PPRCA
Prevalence <1 / 1 000 000
Inheritance Autosomal dominant
or Not applicable
Age of onset All ages
  • H35.5
MeSH -
MedDRA -


Disease definition

Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare, commonly bilateral and symmetric retinal disease characterized by non-progressive or slowly progressive chorioretinal atrophy, peripapillary pigmentary changes and accumulation of ''bone-corpuscle'' pigmentation along the retinal veins and which is usually asymptomatic or can present with mild blurred vision.

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