AIM: To explore the histopathologic changes of Reis-Bücklers corneal dystrophy(RBCD).

METHODS: Cornea buttons were obtained from patients in 1 pedigree who underwent lamella keratoplasty. Sections with HE and special staining which included symplectic blue staining and Masson staining and Congo red staining were observed under light microscope. Two normal cornea specimens(donated corneas from eye bank)were used as control.

RESULTS: In those patients, the bowman's membrane disappeared. The main lesion was in bowman's membrane through HE staining, positive through PAS Congo red staining and Masson staining, negative through symplectic blue staining.

CONCLUSION: The Reis - Bücklers corneal dystrophy is characterized as geographic map-like lesion of bowman's membrane, and the abnormal extracellular deposit is amyloid protein fiber.