By Gary M. White, MD
Phacomatosis Pigmentokeratotica (PPK) is a distinctive new epidermal nevus syndrome characterized by the presence of nevus sebaceous arranged along Blaschko lines, a speckled lentiginous nevus, and skeletal and neurologic abnormalities.
- PPk is a mosaic RASopathy with malignant potential.
- Both activating mutations in KRAS or HRAS may cause PPK.
- A vaginal botryoid rhabdomyosarcoma has been associated [PD 2017;34;352].
It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.