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Phakomatosis pigmentokeratotica

ORPHA:2874· ICD-10 Q85.8

Definition

A rare epidermal nevus disorder characterized by the association of speckled lentiginous nevi with epidermal sebaceous nevi, and extracutaneous anomalies.

Prevalence
<1 / 1 000 000
Inheritance
Unknown
Age of onset
Infancy, Neonatal