Introduction:

Schnyder's crystalline dystrophy is a rare autosomal dominant disease. This disorder causes a stromal dystrophy, characterized by bilateral corneal opacification, resulting from an abnormal accumulation of cholesterol and lipid. UBIAD1 is the causative gene.

Case report:

A male patient aged 49 with a history of progressive visual deterioration since adolescence, hypercholesterolemia and cholecystectomy. On examination it was found both eyes with discoid heterogeneous corneal reticular opacity yellowish-white of well-defined edges with the presence of fine crystals polychromatic and a small area surrounding clear cornea and a lipid arc 360°; the perilimbic area was respected.

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