Abstract
Lipid deposition at the limbus is a feature of familial and non-familial dyslipoproteinemias and can also occur without apparent accompanying systemic abnormality. Hyperlipoproteinemia, most notably type II hyperlipoproteinemia, is frequently associated with bilateral corneal arcus, with less common association in types III, IV and V. Diffuse bilateral opacification of the cornea with accentuation towards the limbus is a feature of HDL deficiency syndromes and LCAT deficiency. Whereas the lipid accumulation of hyperlipoproteinemia may be representative of excessive insudation of lipoprotein from plasma into the cornea that of hypoliproteinemia is more likely to be a consequence of defective lipid clearance. The situation is yet further complicated by the modifying influences of secondary factors, both local and systemic.
Similar content being viewed by others
Article PDF
References
Grundy SM : Pathogenesis of hyperlipoproteinemia. J Lipid Res 1984, 25: 1611–18.
Bolton CH : Lipid disorders. In Holton JB ed. The inherited metabolic diseases, London. Churchill Livingstone 1987: 358–83.
Brown MS and Goldstein JL : Receptor mediated control of cholesterol metabolism. Science 1976, 191: 150–4.
Courtice FC and Garlick DG : The permeability of the capillary wall to the different plasma lipopro-teins of the hypercholesterolemic rabbit in relation to their size. Quart J exp Physiol 1962, 47: 221–7.
Bell FP, Adamson IL, Schwartz CJ : Aortic endo-thelial permeability to albumin: focal and regional patterns of uptake and transmural distribution of I131 albumin in the young pig. Exp Mol Pathol 1974, 20: 57–68.
Friedman M and Byers SO : Some local factors affecting iridic lipid infiltration in hypercholesterolemic rabbits. Am j Physiol 1959, 197: 842–9.
Friedman M and Byers SO : Excess lipid leakage: a property of very young vascular endothelium. Br J exp Path 1962, 43: 363–72.
Walton KW : The role of altered vascular permeability in the induction of experimental xanthomata. Nutr Metab 1973, 15: 59–67.
Rippe B, Kamiya A, Folkow B : Transcapillary passage of albumin, effects of tissue cooling and of increases in filtration and plasma colloid osmotic pressure. Acta Physiol Scand 1979, 105: 171–87.
Zemplenyi T : Enzyme Biochemistry of the arterial wall. London. Lloyd-Luke, 1968.
Cogan DG and Kuwabara T : Lipid keratopathy and atheroma. Circulation 1958, 18: 519–25.
Bron AJ and Easty DL : Fluorescein angiography of the globe and anterior segment. Trans ophthalmol Soc UK 1970, 90: 339–67.
Crispin SM : Lipid Keratopathy in the dog. PhD Thesis University of Edinburgh 1984.
Cogan DG and Kuwabara T : Ocular changes in experimental hypercholesterolemia. Arch Ophthalmol 1959, 61: 219–25.
Meesman A : Anatomischer befund eines auges mit massenhafter ablagerung von cho-lesterinkristallen im vorderen kammer und sekundarer atheromatose der hornhaut. Arch f Augenheilk 1924, 94: 56–72.
Zeeman WPC and Groen J : Lipidosis corneae. Gen-eeskundije bladen uit klinick en laboratorium voor depraktijk 1947, 41: 375–411.
Rodger FC : A study of the ultrastructure and cytochemistry of lipid accumulation and clearance in cholesterol fed rabbit cornea. Exp Eye Res 1971, 14: 88–93.
Landis EM and Pappenheimer JR : Circulation. In Hamilton WF, Dow P eds. Handbook of Physiology, Washington DC: American Physiological Society 1963: Volume 11, Section 2: 961–1034.
Fielder AR, Winder AF, Cooke DE Bowcock SA : Arcus senilis and corneal temperature in man. In Trevor-Roper P ed. Proc Eur Congr Ophthalmol, London: Royal Society of Medicine, 1981: 1015–20.
Fielder AR, Winder AF, Sheraidah GAK, Cooke ED : Problems with corneal arcus. Trans ophthalmol Soc UK 1981, 101: 22–6.
Forsius H and Eriksson A : Pterygium and its relation to arcus senilis, pinguecula and other similar conditions. Acta Ophthalmol 1962, 40, 402–10.
Macaraeg PVJ Jr, Lasagna L, Snyder B : Arcus not so senilis. Ann Intern Med 1968, 68: 345–54.
Winder AF : Relationship between corneal arcus and hyperlipidemia is clarified by studies in familial hypercholesterolemia. Br J Ophthalmol 1981, 67: 789–94.
Spaeth GL : Ocular manifestations of the lipidoses In Tasman W ed. Retinal diseases in children, New York: Harper and Row 1971: 127–206.
Small DM and Shipley GG : Physical-chemical basis of lipid deposition in atherosclerosis. Science 1974, 185: 222–9.
Smith EB : Molecular interactions in human atherosclerotic plaques. Am J Pathol 1977, 86: 665–74.
Small DM : Progression and regression of atherosclerotic lesions. Arteriosclerosis 1988, 8: 103–29.
Camejo G : The interaction of lipids and lipoproteins with the intercellular matrix of arterial tissue; its possible role in atherogeneis. Adv Lipid Res 1982, 19: 1–53.
Borcherding MS, Blacik LJ, Sittig RA, Bizzeil JW, Breen M, Weinstein HG : Proteoglycans and collagen fibre organisation in human corneoslceral tissue. Exp Eye Res 1975, 21: 59–70.
Sheraidah GAK, Winder AF, Fielder AR : Lipid-protein constituents of human corneal arcus. Atherosclerosis 1981, 40: 91–8.
Camejo G, Olofsson S-O, Lopez F, Carlson P, Bondjers G : Identification of apo-B100 segments mediating the action of low density lipoproteins with arterial proteoglycans. Arteriosclerosis 1988, 8: 368–77.
Tschetter RT : Lipid analysis of the human cornea with and without arcus senilis. Arch Ophthalmol 1976, 76: 403–5.
Deckelbaum RJ, Shipley GG, Small DM : Structure and interactions of lipids in human plasma low density lipoproteins. J Biol Chem 1977, 252: 744–54.
Yamashita S, Matsuzawa Y, Kubo M, Tarui S : Coronary heart disease and corneal opacification in hyperalphalipoproteinemia. Arteriosclerosis 1987, 7: 504a.
Beaumont JL, Carlson LA, Cooper GR, Fejfar Z, Fredrickson DS, Strasser T : Classification of hyperlipidemias and hyperlipoproteinemias. Bull WHO 1970, 43: 891–915.
Fredrickson DS, Goldstein JL, Brown MS : The familial hyperlipoproteinemais. In Stansbury JB, Wyngaarden JB, Fredrickson DS eds. The metabolic basis of inherited disease 4th ed. New York. McGraw-Hill 1978: 604–55.
Winder AF : Disorders of lipid and lipoprotein metabolism. In Garner A, Klintworth GK eds. Pathobiology of ocular disease Part B, Switzerland: Marcel Dekker 1982: 1047–75.
Thompson GR, Soutar AK, Spengel FA, Jadhav A, Gavigan S, Myant NB : Defects of the receptor-mediated low density lipoprotein catabolism in homozygous familial hypercholesterolemia and hypothyroidism in vivo. Proc Nat Ac Sci 1981, 78: 2591–2595.
Crispin SM and Barnett KC : Arcus lipoides corneae secondary to hypothroidism in the Alsatian. J Small Anim Pract 1978, 19: 127–12.
Cogan DG and Kuwabara T : Arcus senilis, its pathology and histochemistry. Arch Ophthalmol 1959, 61: 553–60.
Winder AF : Ophthalmological complications of the hypertriglyceridemia. Trans Ophthalmol Soc UK 1980, 100: 119–22.
Vinger PF and Sachs BA : Ocular manifestations of hyperlipoproteinemia. Am J Ophthalmol 1970, 70: 563–73.
Williams HP, Bron AJ, Tripathi RC, Garner A : Hereditary crystalline corneal dystrophy with an associated blood lipid disorder. Trans Ophthalmol Soc UK 1971, 91: 531–42.
Crispin SM : Crystalline stromal dystrophy in the dog. Cornea 1988, 7: 149–61.
Bron AJ : Corneal changes in the dyslipopro-teinemias. Cornea 1988 (in press).
Schaefer EJ : Clinical, biochemical, and genetic features in familial disorders of high density lipoprotein deficiency. Arteriosclerosis 1984, 4: 303–22.
Winder AF and Borysiewicz LK : Corneal opacification and familial disorders affecting plasma high density lipoprotein. Birth Defects Original Article Series 1982, 18: 433–40.
Forte T and Nichols AV : Application of electron microscopy to the study of plasma lipoprotein structure. Adv Lipid Res 1972, 10: 1–41.
Herbert PN, Gotto AM, Fredrickson DS : Familial lipoprotein deficiency. In Stansbury JB, Wyngaarden JB, Fredrickson DS eds. The metabolic basis of inherited disease 4th ed. New York. McGraw-Hill 1978: 544–88.
Katz SS, Small DM, Brook JG, Lees RS : The storage lipids in Tangier disease. J Clin Invest 1977, 59: 1045–54.
Gjone E and Bergaust B : Corneal opacity in familial plasma cholesterol ester deficiency. Acta Ophthalmol 1969, 47: 222–7.
Vrabec MP, Shapiro MB, Koller E, Wiebe DA, Herricks MS, Albers JJ : Ophthalmic observations in lecithin cholesterol acyltransferase deficiency. Arch Ophthalmol 1988, 106: 225–9.
Winder AF, Garner A, Sheraidah GA, Barry P : Familial lecithin: cholesterol acyltransferase deficiency. Biochemistry of the cornea. J Lipid res 1985, 26: 283–7.
Philipson BT : Fish eye disease. Birth Defects Original Article Series 1982, 18: 441–8.
Carlson LA and Philipson BT : Fish Eye Disease, a new familial condition with massive corneal opacities and dyslipoiproteinemia. Lancet 1979, ii: 921–3.
Carlson LA and Holmquist L : Evidence for deficiency of high density lipoiprotein lecithin: cholesterol acyltransferase activity (alpha-LCAT) in Fish Eye Disease. Acta Med Scand 1985, 218: 180–96.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Crispin, S. Lipid deposition at the limbus. Eye 3, 240–250 (1989). https://doi.org/10.1038/eye.1989.33
Issue Date:
DOI: https://doi.org/10.1038/eye.1989.33