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March 1973

Pigmentovascular Nevus

Author Affiliations

Kyoto, Japan

Arch Dermatol. 1973;107(3):463-464. doi:10.1001/archderm.1973.01620180101036

To the Editor.—  Pigmentovascular nevus is characterized by the coexistence of pigmentary nevus and vascular nevus. In our review of the literature, approximately 50 cases of this disorder have been reported, mostly in Japan, by the designation of "phacomatosis pigmentovascularis." Our present case appears to be unique in the association with colon polyposis and its malignant degeneration.

Report of a Case  An 11-year-old Japanese girl was seen because she passed a bloody stool which was first noted 4 months previously. A walnut-sized, smooth-surfaced tumor was palpable by digital examination approximately 10 cm below the orifice of the anus and this was confirmed by examination with a romanoscope. A barium enema revealed multiple polyps in the colon.Examination of the skin showed three kinds of discolored patches over the various parts of the body. A dull red, irregular-shaped nevus flammeus 8 x 12 cm was present on the right side of

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