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Mutilating palmoplantar keratoderma with periorificial keratotic plaques

Disease definition

A hereditary palmoplantar keratoderma characterized by the combination of bilateral mutilating transgredient palmoplantar keratoderma and periorificial keratotic plaques.

ORPHA:659

Classification level: Disorder
  • Synonym(s):
    • Mutilating palmoplantar hyperkeratosis with periorificial keratotic plaques
    • Olmsted syndrome
    • Palmoplantar and periorificial keratoderma
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant or X-linked recessive or Not applicable 
  • Age of onset: Infancy, Neonatal, Childhood
  • ICD-10: Q82.8
  • ICD-11: EC20.30
  • OMIM: 300918  614594  619208
  • UMLS: C0406761
  • MeSH: -
  • GARD: 4075
  • MedDRA: 10068842

  A summary on this disease is available in Español (2015) Français (2015) Italiano (2015) Nederlands (2015) Polski ()

Detailed information

Disease review articles

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.