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.
