色素血管母斑症（高野—Krüger—土肥型）の1例

PHAKOMATOSIS PIGMENTOVASCULARIS

はじめに

　色素血管母斑症Phacomatosis pigmentova—scularisの高野—Krüger—土肥型は，1921年高野^1）がほとんど全身にわたり単純性血管腫および色素性母斑を併発せる1例と題して，その第1例を報告して以来，本邦での記載の明らかな症例は，自験例も含めて34例^1〜33）報告されている（第1表）。

　本病型の臨床像は，蒙古人斑様の青色斑と単純性血管腫の合併であり，一部ではそれぞれが単独で存在し，また一部では両者が混在するとされている。

A case of the Takano-Krüger-Dohi type of this disease in an 11-month-old boy was reported. He showed portwine stains and aberrant mongolian spots over the entire body surface, nevus of Ota on the face, and melanosis bulbi.

There were no abnormalities on physical examination and on results of laboratory tests. No unilateral hyperplasia of the extremity was proved.

Histologic specimen showed melanin granules in the middermis and lower dermis. Dopa reaction showed many positive cells in the cutis.

Statistic studies on 34 reported cases in Japan were performed. They were accompanied with melanosis bulbi and nevus of Ota in 78.6% and 35.3% respectively. These two symptoms belong to the cardinal symptoms as well as portwine stain and aberrant mongolian spot in this disease.