Contributor: Gordon K. Klintworth
The different types of lattice corneal dystrophy are inherited disorders characterized by irregular linear opacities due to the deposition of amyloid in the corneal stroma. Descemet's membrane and the corneal endothelium are unremarkable. In lattice corneal dystrophy type I, lattice corneal dystrophy type II and lattice corneal dystrophy type IIIA amyloid is apparently localized to the cornea, but in lattice corneal dystrophy type II it is a manifestation of a systemic disease. In most types of the lattice corneal dystrophy the amyloid has not been identified. In lattice corneal dystrophy type I it is related to the protein product of the TGFBI gene; in lattice corneal dystrophy type II it is derived from a fragment of mutant gelsolin. Mutations in different parts of the TGFBI gene account for lattice corneal dystrophy type I, lattice corneal dystrophy type IIIA and several other corneal dystrophies (including granular corneal dystrophy, Reis-Bücklers corneal dystrophy and Thiel-Behnke corneal dystrophy).