Phakomatosis pigmentovascularis was first reported in 1947 as a disease complex with a distinct association of pigmentary nevus such as nevus of Ota, nevus of Ito, Mongolian spots, nevus spilus and cutaneous hemangioma such as nevus flammeus,
which
was
associated with systemic organ involvment.
We report a case of phakomatosis pigmentovascularis in a 15-year-old girl who had nevus flammeus, nevus spilus, and nevus of Ota over various parts of her body since birth. She was also associated with leg-length discrepancy, but other systemic
involvement was absent.
(Kor J Dermatol 1994 ; 32(4) : 707~712)
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