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Reis-Bücklers corneal dystrophy

Disease definition

Reis-Bücklers corneal dystrophy (RBCD), also known as granular corneal dystrophy type III, is a rare form of superficial corneal dystrophy characterized by bilateral symmetrical reticular opacities in the superficial central cornea, with progressive visual impairment.

ORPHA:98961

  • Synonym(s):
    • Anterior limiting membrane dystrophy type 1
    • Anterior limiting membrane dystrophy type I
    • Atypical granular corneal dystrophy
    • Corneal dystrophy of Bowman layer type 1
    • Corneal dystrophy of Bowman layer type I
    • Geographic corneal dystrophy
    • Granular corneal dystrophy type 3
    • Granular corneal dystrophy type III
    • RBCD
    • Superficial granular corneal dystrophy
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: H18.5
  • OMIM: 608470
  • UMLS: C0339278
  • MeSH: C535476
  • GARD: 9276
  • MedDRA: -

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