Abstract
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.
Full text
PDF

Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Caputo R., Sambvani N., Monti M., Cavicchini S., Carrassi A., Ratiglia R. Dermochondrocorneal dystrophy (François' syndrome). Report of a case. Arch Dermatol. 1988 Mar;124(3):424–428. [PubMed] [Google Scholar]
- JENSEN V. J. Dermo-chondro-corneal dystrophy; report of a case. Acta Ophthalmol (Copenh) 1958;36(1):71–78. doi: 10.1111/j.1755-3768.1958.tb02261.x. [DOI] [PubMed] [Google Scholar]
- Remky H., Engelbrecht G. Dystrophia dermo-chondro-cornealis (François) Klin Monbl Augenheilkd. 1967;151(3):319–331. [PubMed] [Google Scholar]
- Ruiz-Maldonado R., Tamayo L., Velazquez E. Dystrophie dermo-chondro-cornéenne familiale (syndrome de François). Ann Dermatol Venereol. 1977 Jun-Jul;104(6-7):475–478. [PubMed] [Google Scholar]




