Orphanet: Transgrediens et progrediens palmoplantar keratoderma
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Transgrediens et progrediens palmoplantar keratoderma

Disease definition

A rare, isolated, diffuse palmoplantar keratoderma disorder characterized by red-yellow, moderate to severe hyperkeratosis of the palms and soles, extending to the dorsal aspects of the hands, feet and/or wrists and involving the skin over the Achilles' tendon (transgrediens), gradually worsening with age (progrediens) to include patchy hyperkeratosis over the shins, knees, elbows and, sometimes, skin flexures. Hyperhidrosis is usually associated. Histologically, either epidermolytic or nonepidermolytic changes may be seen.

ORPHA:495

Classification level: Disorder
  • Synonym(s):
    • Greither disease
    • Keratosis extremitatum hereditaria progrediens
    • Keratosis palmoplantaris transgrediens et progrediens
    • Progressive diffuse PPK
    • Progressive diffuse palmoplantar keratoderma
    • Transgrediens et progrediens PPK
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Childhood
  • ICD-10: Q82.8
  • OMIM: 133200
  • UMLS: C1851479  C1851480
  • MeSH: -
  • GARD: 3096
  • MedDRA: -
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