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Mutilating palmoplantar keratoderma with periorificial keratotic plaques

ORPHA:659· ICD-10 Q82.8

Definition

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

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant, Not applicable, X-linked recessive
Age of onset
Childhood, Infancy, Neonatal