舌癌を伴ったCole-Engman症候群の1症例

A Cole-Engman Syndrome with Tongue Cancer

I．はじめに

　Cole-Engman症候群は頸部，躯幹上部，四肢の網状色素沈着，爪の萎縮・変形および粘膜白斑症を主症状とする先天性外胚葉形成異常のきわめて稀な疾患である。本疾患の性格上その報告も皮膚科領域に多く，耳鼻咽喉科における本疾患の報告は1982年に仲田ら^1）が1症例を報告したもののみである。

　このたびわれわれは舌癌を当科で精査，加療中に，その特異な皮膚病変と血液異常，ならびに家族歴より全身性の遺伝性疾患を疑い，最終的にCole-Engman症候群と診断しえた1症例を経験した。その臨床経過を報告するとともに，若干の考察を加えた。

Cole-Engman syndrome is a rare genodermatosis characterized by the triad of reticulated hyperpig-mentation of the skin, nail dystrophy, and leu-koplakia of mucous membrane.

A 39-year-old male was treated for tongue cancer. Hematological examination revealed co-existence of aplastic anemia.

In his family history, his elder brother died of pancytopenia at the age of 28 had hyperpigmen-tation of the skin. As is often the case with this disease, X-linked recessive inheritance was suggest-ed.