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Pigmented paravenous retinochoroidal atrophy

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.


Classification level: Disorder
  • Synonym(s):
    • PPRCA
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: All ages
  • ICD-10: H35.5
  • OMIM: 172870
  • UMLS: C1868310
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


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