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Cutis laxa

Orpha number ORPHA209
Prevalence of rare diseases <1 / 1 000 000
Inheritance
  • Sporadic
  • Autosomal dominant
  • X-linked recessive
  • Autosomal recessive
Age of onset -
ICD 10 code
  • Q82.8
MIM number -
Synonym(s) -

Summary

Cutis laxa is a connective tissue disorder due to deficiencies of elastic fibers. Skin shows hyperlaxity and seems flaccid with folds, especially around the face, conferring a prematurely aged look to the patient. Hereditary forms are usually transmitted as autosomal recessive or dominant traits and associate cutaneous disorders with a variety of visceral lesions. Secondary forms are sometimes associated with non-specific skin eruptions and may be caused by systemic or idiopathic amylosis. Finally some forms of cutis laxa are associated with other connective tissue disorders as in Ehlers-Danlos syndrome or elastic pseudoxanthoma. * Author: C. Blanchet-Bardon, M.D. (April 2002) *

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