Hereditary Corneal Dystrophies

Epithelial corneal dystrophies are Cogan's microcystic dystrophy, Meesman's dystrophy and Reis-Buckler's ring dystrophy [123]. The epithelial dystrophies present with photophobia and/or foreign body sensation. The most common form of epithelial dystrophy is Cogan's microcystic dystrophy, affecting females of middle age. A thickened and folded basement membrane with epithelial cysts containing necrotic debris is characteristic. Bowman's membrane is not involved. In Meesman's dystrophy small layers of the basement membrane can be found between the epithelial cells. Reis-Buckler ring dystrophy is a bilateral, autosomal dominant dystrophy, not only affecting the epithelium, but also the anterior corneal stroma. The epithelium is oedematous and atrophic, Bowman's membrane is interrupted and the anterior stroma contains abnormal fibrous tissue. Histology is not specific and transmission electron microscopy, which will show electron-dense rods in the superficial stroma, is necessary to confirm the diagnosis [87].

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