Orphanet: Phakomatosis pigmentokeratotica

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Phakomatosis pigmentokeratotica

Disease definition

Phakomatosis pigmentokeratotica (PPK) is a very rare epidermal nevus disorder characterized by the association of speckled lentiginous nevi with epidermal sebaceous nevi, and extracutaneous anomalies.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: <1 / 1 000 000
  • Inheritance: Unknown 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q85.8
  • OMIM: -
  • UMLS: C2931658
  • MeSH: C537893
  • GARD: 4311
  • MedDRA: -
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