Dermochondral corneal dystrophy (of François).
Dermochondral corneal dystrophy (of François) has been reported rarely in the literature. It consists of a triad of findings characterised by the development of skin nodules, acquired deformities of the extremities, and a corneal dystrophy. The corneal dystrophy is central and superficial with whitish subepithelial opacities. We present two brothers who display previously unreported ocular findings. Specifically, they developed confluent opacification of their central corneas with anterior stromal involvement, and peculiar anterior cortical cataracts. These findings should be added to the spectrum of findings seen in this rare disorder.
Bierly
J R
JR
Francis Proctor Foundation, University of California, San Francisco 94143.
George
S P
SP
Volpicelli
M
M
D000328:Adult; D002386:Cataract / Q000209:etiology; D003317:Corneal Dystrophies, Hereditary / Q000150:complications*; D003318:Corneal Opacity / Q000209:etiology; D005531:Foot Deformities, Acquired / Q000150:complications*; D006227:Hand Deformities, Acquired / Q000150:complications*; D006801:Humans; D008297:Male; D012871:Skin Diseases / Q000150:complications*