A Case of Phakomatosis Pigmentovascularis with Eye Involvement / 대한피부과학회지

ABSTRACT

Phakomatosis pigmentova.scularis is a disease complex with dermal melanocyto sis such as nevus of Ota, nevus of Ito, or Mongolian spots and extensive hemangiomatous change such as nevus flammeus, which are associated with systemic organ involvement. We report a 23-year-old female, who showed bilateral nevus of Ota and nevus of Ito with widespread nevus flammeus and Mongolian spots since birth. She was also associated with megalocornea due to congenitol glaucoma and cataract.