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Mutilating palmoplantar keratoderma with periorificial keratotic plaques

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
  • Q82.8
  • C2609071
MeSH -
  • 10068842


Disease definition

Mutilating palmoplantar keratoderma (PPK) with periorificial keratotic plaques (also known as Olmsted syndrome) is a hereditary palmoplantar keratoderma characterized by the combination of bilateral mutilating transgredient palmoplantar keratoderma and periorificial keratotic plaques.

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