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Bietti crystalline dystrophy

Disease definition

A rare progressive autosomal recessive tapetoretinal degeneration disease, occurring in the third decade of life, characterized by small sparkling crystalline deposits in the posterior retina and corneal limbus in addition to sclerosis of the choroidal vessels and manifesting as nightblindness, decreased vision, paracentral scotoma, and, in the end stages of the disease, legal blindness.


Classification level: Disorder
  • Synonym(s):
    • BCD
    • Bietti crystalline corneoretinal dystrophy
    • Bietti crystalline retinopathy
  • Prevalence: Unknown
  • Inheritance: Autosomal recessive 
  • Age of onset: -
  • ICD-10: H35.5
  • ICD-11: 9B61
  • OMIM: 210370
  • UMLS: C1859486
  • MeSH: C535440
  • GARD: 10050
  • MedDRA: -

Detailed information

Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.