基底細胞母斑症候群の2症例

Basal Cell Nevus Syndrome: Report of Two Cases

　はじめに

　基底細胞母斑症候群は，母斑などの皮膚症状，脊椎や肋骨などの骨格異常，多発性顎嚢胞などを主症状とする遺伝性疾患であり，本邦においてはおもに皮膚科，口腔外科領域で報告されてきた。

　最近われわれは本症候群と考えられる母子の2症例を経験したので報告する。

Basal cell nevus syndrome has rarely been re-ported in the otolaryngological literature. The syndrome consists principally of multiple basal cell nevi, cysts of the jaws and skeletal anomalies.

Two cases of basal cell nevus syndrome arc presented and the previous literature is reviewed. The first case, a 40-year-old female, showed mu-ltiple maxillary cysts, palmar and plantar pits, scoliosis and mild hypertelorism. The other case, a 14-year-old male, was the third son of the first case and presented with multiple cysts of the jaw. On examination, palmar and plantar pits and multiple jaw cysts with impacted teeth were noted.