Dermato-chondro-corneal dystrophyQ87.8

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)

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HistoryThis section has been translated automatically.

François, 1949

DefinitionThis section has been translated automatically.

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

LocalizationThis section has been translated automatically.

Infancy

Clinical featuresThis section has been translated automatically.

hands and feet

LaboratoryThis section has been translated automatically.

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

LiteratureThis section has been translated automatically.

  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

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer