Synonyms
“Avellino” corneal dystrophy; GCDII
Definition and Characteristics
Large rings with punched-out centers, powdery disks or stellate elements in the center of the cornea dominate the picture in this autosomal-dominantly inherited CD. The rings and disks are located in the superficial stroma, whereas the stars extend to the deeper stroma. These opacities are usually so few in number that they can be counted. The peripheral cornea is free of opacifications. The dystrophy manifests in the second decade of life, progression is delayed about 20–30 years and is characterized more by individual growth than by increase in number of opacities. In a subsequent stage, some units enlarge, creating a “sword-like” or “christmas-tree” appearance. Rarely, coalescing rings and disks create ground-glass haziness in a third stage of the disease. Recurrent painful attacks due to epithelial erosions occur less frequently in GCDII than in GCDI. The homozygous form of GCDII presents already in...
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References
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Lisch, W., Janecke, A., Seitz, B. (2009). Corneal Dystrophy, Granular Type II. In: Lang, F. (eds) Encyclopedia of Molecular Mechanisms of Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-29676-8_3307
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DOI: https://doi.org/10.1007/978-3-540-29676-8_3307
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-67136-7
Online ISBN: 978-3-540-29676-8
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