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[Familial dermo-chondro-corneal dystrophy (François' syndrome)].

1977; National Institutes of Health; Volume: 104; Issue: 6-7 Linguagem: Inglês

Ramón Ruiz‐Maldonado, Lourdes Tamayo, Eridán González Velázquez,

Connective tissue disorders research

The François' syndrome associates disseminated firm nodular subcutaneous lesions, a deforming arthropathy and a corneal dystrophy. Inheritance seems to be recessive. Six cases of this rare syndrome have been previously published. The syndrome is reported here in two Mexican brothers. In the authors' opinion the François' syndrome is not related to the xanthomatoses. It should be considered as a genetically recessive arthropathic nodular fibromatosis.