Cover

Corneal Dystrophies

Editor(s): Lisch W. (Hanau) 
Seitz B. (Homburg/Saar) 
Table of Contents
Vol. 48, 2011
Section title: Paper
Lisch W, Seitz B (eds): Corneal Dystrophies. Dev Ophthalmol. Basel, Karger, 2011, vol 48, pp 67–96
(DOI:10.1159/000324078)

Differential Diagnosis of Schnyder Corneal Dystrophy

Weiss J.S. · Khemichian A.J.
aDepartment of Ophthalmology, Louisiana State University Health Science Center, New Orleans, La.; bKresge Eye Institute, Wayne State University School of Medicine, Detroit, Mich., USA

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Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 4/26/2011
Cover Date: 2011

Number of Print Pages: 30
Number of Figures: 6
Number of Tables: 0

ISBN: 978-3-8055-9720-3 (Print)
eISBN: 978-3-8055-9721-0 (Online)

Abstract

Schnyder corneal dystrophy (SCD) is a rare corneal dystrophy characterized by abnormally increased deposition of cholesterol and phospholipids in the cornea leading to progressive vision loss. SCD is inherited as an autosomal dominant trait with high penetrance and has been mapped to the UBIAD1 gene on chromosome 1p36.3. Although 2/3 of SCD patients also have systemic hypercholesterolemia, the incidence of hypercholesterolemia is also increased in unaffected members of SCD pedigrees. Consequently, SCD is thought to result from a local metabolic defect in the cornea. The corneal findings in SCD are very predictable depending on the age of the individual, with initial central corneal haze and/or crystals, subsequent appearance of arcus lipoides in the third decade and formation of midperipheral haze in the late fourth decade. Because only 50% of affected patients have corneal crystals, the International Committee for Classification of Corneal Dystrophies recently changed the original name of this dystrophy from Schnyder crystalline corneal dystrophy to Schnyder corneal dystrophy. Diagnosis of affected individuals without crystalline deposits is often delayed and these individuals are frequently misdiagnosed. The differential diagnosis of the SCD patient includes other diseases with crystalline deposits such as cystinosis, tyrosinemia, Bietti crystalline dystrophy, hyperuricemia/gout, multiple myeloma, monoclonal gammopathy, infectious crystalline keratopathy, and Dieffenbachia keratitis. Depositions from drugs such as gold in chrysiasis, chlorpromazine, chloroquine, and clofazamine can also result in corneal deposits and are different from SCD. Diseases of systemic lipid metabolism that cause corneal opacification, such as lecithin-cholesterol acyltransferase deficiency, fish eye disease and Tangier disease, should also be considered although these are autosomal recessive disorders.


  

Author Contacts

Jayne S. Weiss, MD, Chair of Department of Ophthalmology Herbert Kaufman, Professor of Ophthalmology Louisiana State University Health Science Center 2020 Gravier Street, New Orleans, LA 70112 (USA) E-Mail jayneweiss@aol.com

  

Article Information

Published online: April 26, 2011
Number of Print Pages : 30

  

Publication Details

Book Serie: Developments in Ophthalmology, Vol. 48, Year 2011

Editor(s): Bandello, F. (Milan)
ISSN: 0250-3751 (Print), eISSN: 1662-2790 (Online)

For additional information:
http://content.karger.com/ProdukteDB/produkte.asp?issn=0250-3751

Book Title: Corneal Dystrophies

Editor(s): Lisch W, Seitz B (eds)

For additional information:
http://content.karger.com/ProdukteDB/produkte.asp?issn=0250-3751&volume=48


Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 4/26/2011
Cover Date: 2011

Number of Print Pages: 30
Number of Figures: 6
Number of Tables: 0

ISBN: 978-3-8055-9720-3 (Print)
eISBN: 978-3-8055-9721-0 (Online)


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