SUBEPITHELIAL MUCINOUS CORNEAL-DYSTROPHY - CLINICAL AND PATHOLOGICAL CORRELATIONS

We describe a family with an unusual autosomal dominant anterior corne al dystrophy. The onset was characterized by frequent, recurrent corne al erosions in the first decade. This subsided during adolescence and was followed by progressive decreased vision. Slit-lamp examination re vealed bilateral subepithelial opacities and haze, involving the entir e cornea, but most dense centrally. Histopathological study revealed a subepithelial band of eosinophilic, periodic acid-Schiff-positive, al cian blue-positive, hyaluronidase-sensitive material anterior to Bowma n's layer. Electron microscopy demonstrated subepithelial deposition o f fine fibrillar material consistent with glycosaminoglycan. Immunohis tochemical analysis indicated that the accumulated material contained a combination of chondroitin 4-sulfate and dermatan sulfate. This uniq ue condition clinically resembled Grayson-Wilbrandt dystrophy, but dif fered histochemically. To our knowledge, this anterior corneal dystrop hy has not previously been reported, and it is best described by the n ame ''subepithelial mucinous corneal dystrophy.''