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Clinicopathologic Reports, Case Reports, and Small Case Series
April 2006

Two Cases of Reis-Bücklers Corneal Dystrophy (Granular Corneal Dystrophy Type III) Caused by Spontaneous Mutations in the TGFBI Gene

Arch Ophthalmol. 2006;124(4):589-593. doi:10.1001/archopht.124.4.589

Reis-Bücklers corneal dystrophy (RBCD) is an inherited corneal disorder that was first described by Reis1 in 1917 and later by Bücklers2 in 1949. Affected individuals have an onset early in life and have frequently recurring, painful corneal erosions, superficial corneal opacities, and significant visual impairment. The literature on this entity, which has several synonyms (granular corneal dystrophy [GCD] type III, superficial variant of GCD, corneal dystrophy of Bowman layer type I), is bewildering not only because of the nomenclature but also because this genetically determined disorder has been confused with a different condition, Thiel-Behnke corneal dystrophy (TBD). Although RBCD and TBD are now considered distinct clinicopathologic disorders,3 a precise diagnosis of these corneal disorders was difficult until recently because it relied only on the clinical and histopathologic features.

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