Abstract To the Editor. — Mucopolysaccharides constitute less than 1% of the dry weight of the sclera and are distributed diffusely between collagen fibers.' Abnormal focal accumulation of scleral mucin is exceptionally rare. Conn and associates,2 in 1982, described for the first time a condition in which bilateral focal scleral accumulation of mucopolysaccharide was associated with retinal pigment epithelial and neurosensory retinal degeneration. The cause of the focal deposits of mucopolysaccharide was unknown and was not related to any systemic metabolic abnormality. The authors proposed that the retinal degeneration was the result of compression of the choriocapillaris by thickened sclera. They called this disorder scleropachynsis maculopathy and considered it analogous to macular corneal dystrophy, although the disorder had no known hereditary basis and the staining characteristics of the mucopolysaccharide in macular dystrophy is somewhat different. To my knowledge, no other case of focal mucopolysaccharide accumulation in the sclera has been References 1. Dische J: Biochemistry of connective tissues of the vertebrate eye . Int Rev Connect Tissue Res 1970;5:209-274. 2. Conn H, Green WR, de la Cruz AC, et al: Scleropachynsis maculopathy . Arch Ophthalmol 1982;100:793-799.Crossref 3. 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 4. Cook HC: Carbohydrates , in Bancroft JD, Steven A, Dawson IMP (eds): Theory and Practice of Histological Technique . New York, Churchill Livingstone Inc, 1977, pp 141-167. 5. Hoffman P, Meyer K, Linker A: Transglycosilation during mixed digestion of hyaluronic acid and chondroitin sulfate by testicular hyaluronidase . J Biol Chem 1956;219:653-663. 6. Johnson WC, Helwig EB: Cutaneous focal mucinosis . Arch Dermatol 1966;93:13-20.Crossref
Archives of Ophthalmology – American Medical Association
Published: Jun 1, 1986
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