TGFBI Corneal Dystrophies: Thiel-Behnke Corneal Dystrophy (TBCD)

Thiel-Behnke corneal dystrophy (TBCD) is an autosomal dominantly inherited stromal corneal dystrophy associated with an R555Q (Arg555Gln) mutation in the TGFBI gene on chromosome 5q31. In 1967, Thiel and Behnke first described this corneal dystrophy, in which corneal deposits accumulate primarily in Bowman’s layer (Thiel and Behnke 1967). The deposits are different than those of Reis-Bücklers corneal dystrophy (RBCD). TBCD was also called as Grayson-Wilbrandt corneal dystrophy and corneal dystrophy Bowman 2 (CDB2) at one time, but these terms are no longer used.