Korean J Dermatol.  1985 Feb;23(1):112-119.

Four Cases of Phakomatosis Pigmentovascularis

• Youn JI
• Joh GY
• Lee AY
• Lee YS

Abstract

Dermal melanocytosis such as nevus of Ota and extensive Mongolian spots are sometimes associated with hemangiomatous changes. Ota gave the designation of phakorriatosis pigmentovascularis to a spectrum of these combinations. We oLserved four cases of phakomatosis pigmentovascularis. They showed extensive, various dermal melanocytosis and widespread nevus flammeus. They exhibited signs of involvement of the systemic organs such as abnormality of electroencephalograrn, soft tissue hypertrophy and convulsion.

Keyword

Phakomatosis pigmentovascularis; Dermal melanocytosis; Nevs flammeus

MeSH Terms

Hypertrophy
Mongolian Spot
Neurocutaneous Syndromes*
Nevus of Ota
Port-Wine Stain
Seizures