典型的な臨床所見を呈した基底細胞母斑症候群の1例

Basal Cell Nevus Syndrome:Report of a Case

　はじめに

　基底細胞母斑症候群（basal cell nevus syn-drome）は多発性顎嚢胞，多発性基底細胞母斑，骨格系の異常を主症状とする常染色体優性遺伝性疾患とされている。本症候群は白人に多く有色人種には比較的少ない疾患と考えられている^1）。今回われわれは，本症候群の典型例と思われる症例を経験したので，文献的考察を加えて報告する。

A 13-year-old male complained of a swelling in the right mandibular region. On physical examina-tion, multiple nevi on the upper trunk and mild hypertelorism were noted. Radiological examina-tions releaved the abnormal features of intracranial calcifications, multiple jaw cysts and bifid ribs. Therefore, typical basal cell nevus syndrome was diagnosed. Multiple jaw cysts were enucleated and pathologically diagnosed as odontogenic kerato-cysts.