Korean J Dermatol.  1989 Jun;27(3):320-324.

A Case of Phakomatosis Pigmentovascularis with Eye Involvement

• Rhee JK
• Ko IJ
• Kim HO
• Kim CW

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.

Keyword

Dermal melanocytosis; Megalocornea; Nevus flammeus Phakomatosis pigmentov ascularis

MeSH Terms

Cataract
Female
Glaucoma
Humans
Mongolian Spot
Neurocutaneous Syndromes*
Nevus
Nevus of Ota
Parturition
Port-Wine Stain
Young Adult