Bierly J R, George S P, Volpicelli M
Francis Proctor Foundation, University of California, San Francisco 94143.
Br J Ophthalmol. 1992 Dec;76(12):760-1. doi: 10.1136/bjo.76.12.760.
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.
（弗朗索瓦）皮肤软骨性角膜营养不良在文献中鲜有报道。它由三联征组成，其特征为皮肤结节形成、肢体后天性畸形以及角膜营养不良。角膜营养不良位于中央且表浅，有白色上皮下混浊。我们报告了两兄弟，他们有此前未报道过的眼部表现。具体而言，他们中央角膜出现融合性混浊并累及前基质，还有特殊的前皮质性白内障。这些表现应补充到这种罕见疾病的表现谱中。
