Thiel-Behnke dystrophies;
Corneal dystrophy of Bowman layer type II;
Honeycomb corneal dystrophy;
Anterior limiting membrane dystrophy type II;
Curly fibers corneal dystrophy;
Waardenburg-Jonker corneal dystrophy

Thiel-Behnke dystrophy (TBCD) is an autosomal dominant dystrophy with recurrent corneal erosions developing in the first and second decade of life. TBCD shares many similarities with Reis-Bucklers corneal dystrophy (RBCD), but decreasing visual acuity is seen later than with RBCD. Electron microscopy reveals curly fibers deposited in Bowman's layer and this finding differentiates it from RBCD. Both TBCD and RBCD are linked to mutations in the TGFBI gene. TBCD is also linked to a mutation on chromosome 10q23-24, the gene product is currently unknown.

Nervous system disease

TGFBI [HSA:7045] [KO:K19519]

Reis-Bucklers corneal dystrophy (RBCD) is described in [DS:H00951]

PMID:19236704

PMID:18214787

PMID:16900028