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August 1993

Subepithelial Mucinous Corneal Dystrophy: Clinical and Pathological Correlations

Author Affiliations

From the Departments of Ophthalmology (Drs Feder, Jay, Stock, and O'Grady) and Pathology (Dr Roth), Northwestern University Medical School, Chicago, Ill, and the Department of Ophthalmology, College of Medicine, University of Illinois at Chicago (Dr Yue).

Arch Ophthalmol. 1993;111(8):1106-1114. doi:10.1001/archopht.1993.01090080102025

We describe a family with an unusual autosomal dominant anterior corneal dystrophy. The onset was characterized by frequent, recurrent corneal erosions in the first decade. This subsided during adolescence and was followed by progressive decreased vision. Slit-lamp examination revealed bilateral subepithelial opacities and haze, involving the entire cornea, but most dense centrally. Histopathological study revealed a subepithelial band of eosinophilic, periodic acid-Schiff-positive, alcian blue-positive, hyaluronidase-sensitive material anterior to Bowman's layer. Electron microscopy demonstrated subepithelial deposition of fine fibrillar material consistent with glycosaminoglycan. Immunohistochemical analysis indicated that the accumulated material contained a combination of chondroitin 4-sulfate and dermatan sulfate. This unique condition clinically resembled Grayson-Wilbrandt dystrophy, but differed histochemically. To our knowledge, this anterior corneal dystrophy has not previously been reported, and it is best described by the name "subepithelial mucinous corneal dystrophy."