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March 1988

Dermochondrocorneal Dystrophy (François' Syndrome): Report of a Case

Author Affiliations

From the Institute of I Dermatologic Clinic and Pediatric Dermatology (Drs Caputo, Sambvani, Monti, and Cavicchini) and the Oculistic Clinic (Dr Ratiglia), University of Milan (Italy); and the Odontologic Clinic, S. Paolo Hospital, Milan (Dr Carrassi).

Arch Dermatol. 1988;124(3):424-428. doi:10.1001/archderm.1988.01670030090029

• Dermochondrocorneal dystrophy (François' syndrome) is an extremely rare disorder characterized by firm, nodular lesions involving the hands and the face; osteochondrodystrophy of the peripheral extremital bones, resulting in limitation of movement; and corneal dystrophy marked by white or brownish opacities. A nonfamilial case of dermochondrocorneal dystrophy was studied in a 45-year-old woman who had severe involvement of the gingival and palatal mucous membranes.

(Arch Dermatol 1988;124:424-428)

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