A new, band-shaped and whorled microcystic dystrophy of the corneal epithelium.
Five family members and three unrelated patients (four women, four men, 23 to 71 years old) had a dystrophy of the corneal epithelium. Direct slit-lamp examination showed bilateral or unilateral, gray, band-shaped, and feathery opacities that sometimes appeared in whorled patterns. Retroillumination showed intraepithelial, densely crowded, clear microcysts. Light and electron microscopy disclosed diffuse vacuolization of the cytoplasm of epithelial cells in the affected area. Visual acuity was so reduced in three patients that abrasion of the corneal epithelium was performed. The corneal abnormalities recurred within months, with the same reduction in visual acuity as before. The corneal opacities were progressive in two patients but diminished noticeably in another after he began using a hard contact lens. We found no other ophthalmic irregularities or associated systemic abnormalities and no indication of drug-induced keratopathy.
Lisch
W
W
Eye Clinic of Hanau, Germany.
Steuhl
K P
KP
Lisch
C
C
Weidle
E G
EG
Emmig
C T
CT
Cohen
K L
KL
Perry
H D
HD
D000328:Adult; D000368:Aged; D003317:Corneal Dystrophies, Hereditary / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D003318:Corneal Opacity / Q000473:pathology / Q000601:surgery; D003560:Cysts / Q000473:pathology*; D004848:Epithelium / Q000473:pathology / Q000601:surgery; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012008:Recurrence; D014617:Vacuoles / Q000473:pathology; D014792:Visual Acuity; D014960:X Chromosome