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Report of a Case

Ruggero Caputo, MD; Niccla Sambvani, MD; Marcello Monti, MD; Stefano Cavicchini, MD; Antonio Carrassi, MD; Roberto Ratiglia, MD

Arch Dermatol. 1988;124(3):424-428.

Abstract
• 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)

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).

Footnotes
Accepted for publication July 12, 1987.

Presented in part as a poster at the meeting of the American Academy of Dermatology, New Orleans, Dec 7, 1986.

Reprint requests to Institute of I Dermatologic Clinic and Pediatric Dermatology, University of Milan, Via Pace 9, 20122 Milan, Italy (Dr Caputo).

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