Unesterified cholesterol in Schnyder's corneal crystalline dystrophy.
We examined a 51-year-old woman who had bilateral corneal crystalline deposits unassociated with xanthelasma or systemic involvement. Her son had similar corneal lesions. Plasma cholesterol and apolipoprotein A-I and B levels were normal in both patients. Histopathologic examination of the corneal button in the mother obtained at the time of keratoplasty disclosed lipid deposits that stained with oil red O as well as with filipin, a fluorescent probe that specifically detects unesterified cholesterol. Electron microscopy showed abnormal accumulation of lipid and cholesterol in the central and paracentral basal epithelium, Bowman's layer, and superficial stroma.
Rodrigues
M M
MM
Kruth
H S
HS
Krachmer
J H
JH
Willis
R
R
D002784:Cholesterol / Q000378:metabolism*; D003315:Cornea / Q000378:metabolism / Q000648:ultrastructure; D003317:Corneal Dystrophies, Hereditary / Q000378:metabolism* / Q000628:therapy; D016039:Corneal Transplantation; D003460:Crystallization; D005260:Female; D005372:Filipin; D006801:Humans; D008854:Microscopy, Electron; D008875:Middle Aged
D005372:Filipin; D002784:Cholesterol