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Lattice corneal dystrophy type I is one of the more common corneal dystrophies and occurs throughout the world. Randomly oriented linear opacities resembling cotton threads accumulate in the central portions of the stroma. These usually become apparent in the first decade of life although they are sometimes seen in infancy. The peripheral cornea is relatively spared and intervening stromal areas are clear. This is a progressive disorder in which vision during childhood is often normal but by the fifth and sixth decades most patients have severe visual impairment due to increasing accumulations of amyloid. Corneal erosions may occur in the absence of stromal infiltrates.
No systemic disease is found in LCD1 (as opposed to LCD type II).
Type I lattice dystrophy is an autosomal dominant disorder as the result of mutations in the TGFBI gene (5q31). Other corneal dystrophies (granular I or Groenouw type I, combined granular/lattice or Avellino type, Thiel-Behnke, Reis-B?ocklers, epithelial basement membrane disease) have mutations in the same region of the same gene casting doubt on the value of using solely clinical and histologic distinctions in current classifications of these corneal disorders.
Recurrent corneal erosions benefit from standard treatments while penetrating keratoplasty may be necessary by the fifth decade to improve acuity.