Cholesterol localization in ultrathin frozen sections in Schnyder's corneal crystalline dystrophy

We examined a 57-year-old woman who had bilateral corneal crystalline deposits associated with xanthelasma. The patient's son had bilateral stromal haze. Plasma cholesterol and apolipoprotein A-I and B levels were normal. Histopathologic examination disclosed lipid deposits, particularly in the superficial stroma and Bowman's layer. These deposits stained with oil red O and filipin, a fluorescent probe that specifically detects unesterified cholesterol. Cryoultramicrotomy disclosed more specific ultrastructural localization of unesterified cholesterol with an array of crystals resembling multiple plates in extracellular corneal tissue.