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Keratosis linearis-ichthyosis congenita-sclerosing keratoderma syndrome

Disease definition

Keratosis linearis-ichthyosis congenita-sclerosing keratoderma syndrome is an inherited epidermal disorder characterized by palmoplantar keratoderma, linear hyperkeratotic papules on the flexural side of large joints (cord-like distribution around wrists, in antecubital and popliteal folds), hyperkeratotic plaques (on neck, axillae, elbows, wrists, and knees), mild ichthyosiform scaling, and sclerotic constrictions around fingers that present flexural deformities.

ORPHA:281201

  • Synonym(s):
    • KLICK syndrome
  • Prevalence: Unknown
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q82.8
  • OMIM: 601952
  • UMLS: C1866029
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

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