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Scleropachynsis Maculopathy

Scleropachynsis Maculopathy Abstract • To our knowledge, this is the first clinicopathologic report of a previously unrecognized maculopathy in which there is thickening and mucopolysaccharide deposition in the sclera subjacent to the macula. A healthy 50-year-old man had bilateral mottling of the retinal pigment epithelium (RPE) in the macular region. The left eye was enucleated because of a choroidal melanoma. Histopathologic examination disclosed a thickened sclera with abnormal collagen fibrils with diameters up to 5,800 A and compressed choroid. The overlying RPE had areas of hypopigmentation, hyperpigmentation, and hyperplasia and contained intracytoplasmic lipofuscin. References 1. Spicer SS, Horn RG, Leppi TJ: Histochemistry of connective tissue mucopolysaccharides , in Wagner BM, Smith DE (eds): The Connective Tissue . Baltimore, Williams & Wilkins Co, 1967, pp 251-303. 2. McKusick VA: Heritable Disorders of Connective Tissue , ed 4. St Louis, CV Mosby Co, 1972, pp 629-630. 3. Brunning RD: Morphologic alterations in nucleated blood and marrow cells in genetic disorders . Hum Pathol 1970;1:99-124.Crossref 4. Scott JE, Quintarelli G, Dellovo MC: The chemical and histochemical properties of Alcian blue: I. The mechanism of Alcian blue staining . Histochemistry 1964;4:73-85.Crossref 5. Garner A: Histochemistry of corneal macular dystrophy . Invest Ophthalmol Vis Sci 1969;8:475-483. 6. Sero N, Meyer K, Anderson B, et al: Variations in keratosulfate . J Biochem 1965;240:1005-1010. 7. Okada S, O'Brien JS: Generalized gangliosidosis: β-Galactosidase deficiency . Science 1968;160:1002-1004.Crossref 8. MacBrinn MC, Okada S, Ho MW, et al: Generalized gangliosidosis: Impaired cleavage of galactose from a mucopolysaccharide and a glycoprotein . Science 1969;163:946-947.Crossref 9. Hoffman P, Meyer K, Linker A: Transglycosilation during the mixed digestion of hyaluronic acid and chondroitin sulfate by testicular hyaluronidase . J Biol Chem 1956;219:653-663. 10. Howard GM: Obscure scleral thickening. Read before the Eastern Ophthalmic Pathology Society Meeting, Philadelphia, April 12, 1971. 11. Virgilio LA, Williams RJ, Klintworth GK: An unusually large human eye with abnormal scleral collagen . Arch Ophthalmol 1976;94:101-105.Crossref 12. Mann I: The Development of the Human Eye , ed 3. New York, Grune & Stratton Inc, 1969, pp 254-255. 13. Duke-Elder S: System of Ophthalmology . St Louis, CV Mosby Co, pt 1, 1913, vol 3, pp 162-163. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

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References (10)

Publisher
American Medical Association
Copyright
Copyright © 1982 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1982.01030030797015
Publisher site
See Article on Publisher Site

Abstract

Abstract • To our knowledge, this is the first clinicopathologic report of a previously unrecognized maculopathy in which there is thickening and mucopolysaccharide deposition in the sclera subjacent to the macula. A healthy 50-year-old man had bilateral mottling of the retinal pigment epithelium (RPE) in the macular region. The left eye was enucleated because of a choroidal melanoma. Histopathologic examination disclosed a thickened sclera with abnormal collagen fibrils with diameters up to 5,800 A and compressed choroid. The overlying RPE had areas of hypopigmentation, hyperpigmentation, and hyperplasia and contained intracytoplasmic lipofuscin. References 1. Spicer SS, Horn RG, Leppi TJ: Histochemistry of connective tissue mucopolysaccharides , in Wagner BM, Smith DE (eds): The Connective Tissue . Baltimore, Williams & Wilkins Co, 1967, pp 251-303. 2. McKusick VA: Heritable Disorders of Connective Tissue , ed 4. St Louis, CV Mosby Co, 1972, pp 629-630. 3. Brunning RD: Morphologic alterations in nucleated blood and marrow cells in genetic disorders . Hum Pathol 1970;1:99-124.Crossref 4. Scott JE, Quintarelli G, Dellovo MC: The chemical and histochemical properties of Alcian blue: I. The mechanism of Alcian blue staining . Histochemistry 1964;4:73-85.Crossref 5. Garner A: Histochemistry of corneal macular dystrophy . Invest Ophthalmol Vis Sci 1969;8:475-483. 6. Sero N, Meyer K, Anderson B, et al: Variations in keratosulfate . J Biochem 1965;240:1005-1010. 7. Okada S, O'Brien JS: Generalized gangliosidosis: β-Galactosidase deficiency . Science 1968;160:1002-1004.Crossref 8. MacBrinn MC, Okada S, Ho MW, et al: Generalized gangliosidosis: Impaired cleavage of galactose from a mucopolysaccharide and a glycoprotein . Science 1969;163:946-947.Crossref 9. Hoffman P, Meyer K, Linker A: Transglycosilation during the mixed digestion of hyaluronic acid and chondroitin sulfate by testicular hyaluronidase . J Biol Chem 1956;219:653-663. 10. Howard GM: Obscure scleral thickening. Read before the Eastern Ophthalmic Pathology Society Meeting, Philadelphia, April 12, 1971. 11. Virgilio LA, Williams RJ, Klintworth GK: An unusually large human eye with abnormal scleral collagen . Arch Ophthalmol 1976;94:101-105.Crossref 12. Mann I: The Development of the Human Eye , ed 3. New York, Grune & Stratton Inc, 1969, pp 254-255. 13. Duke-Elder S: System of Ophthalmology . St Louis, CV Mosby Co, pt 1, 1913, vol 3, pp 162-163.

Journal

Archives of OphthalmologyAmerican Medical Association

Published: May 1, 1982

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