Corneal Dystrophy, Reis-Bücklers
Synonyms
Granular corneal dystrophy type III; GCDIII; RBCD
Definition and Characteristics
RBCD is an autosomal dominantly inherited disorder characterized by subepithelial and superficial stromal alterations extending almost to the limbus. It presents painful erosions in infancy and is associated with fine, granular opacities that spread and become geographic-like with time. The geographic-like opacities are to be regarded as a landmark of RBCD. The full stage of RBCD can be observed in the first decade combined with blurred vision.
Prevalence
RBCD is a rare superficial corneal disorder presented first by Bücklers in 1949 [1]. Weidle described a Caucasian RBCD family with a total of 30 patients in 7 generations [2]. Other reports of true RBCD cases are from France, The Netherlands, USA, Germany, and Japan. RBCD has been frequently confused in the literature with Thiel-Behnke corneal dystrophy (TBCD), which is characterized by honeycomb corneal appearance [2].
Genes
A locus of RBCD has...
References
- 1.Bücklers M (1949) Über eine weitere familiäre Hornhautdystrophie (Reis). Klin Mbl Augenheilk 114:386–397Google Scholar
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