Dermato-chondro-corneal dystrophy Q87.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

dermato-chondro-corneal dystrophy; Dermochondral corneal dystrophy (of Francois); Dystrophia dermo-chondro-cornealis familiaris; François syndrome I; hereditary idiopathic; Idiopathic Osteolysis Type V; Osteolysis; Type V (François)

History
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François, 1949

Definition
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Rare, autosomal recessive hereditary special form of idiopathic osteolysis with characteristic corneal opacities and xanthomas.

Localization
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Infancy

Clinical features
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hands and feet

Laboratory
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Progressive osteolysis of the hands and feet, which initially manifests itself in infancy with sensitivity to touch and difficulty walking, deformation of the fingers and toes, contractures, corneal opacities, xanthomas of the skin

Literature
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  1. Francois J (1949) Dystrophy dermo-chondro-corneenne familiale. Ann Oculist 182: 409-442
  2. Francois J, Detrait C (1950) Dystrophy dermo-chondro-corneenne familiale. Ann Paediat 174: 145-174
  3. Sardella A et al (1998) Gingival lesions in a patient with dermochondrocorneal dystrophy (Francois syndrome). A case report. J Clin Periodontol 25: 1047-1049

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Last updated on: 29.10.2020