Cole-Engman症候群

COLE-ENGMAN'S SYNDROME

はじめに

　Dyskeratosis congenita cum pigmentatione，dystrophia unguium et leukokeratosis orisは先天的外胚葉形成異常の極めて稀な一表現であり，その完全型はこれまでに39例，ことに我国では5例の報告をみるのみで，これに不全型を加えても53例にすぎない。最近，我々は本症の完全型に真性尿崩症とその他の2,3の症候を合併した1例を経験したので，これに蒐集した文献例の統計を加えて報告する。

A 33-year-old man noticed a typical reticulated pigmentation and disturbed growth of the nails 8 years ago. After that time the leukoplakia on the oral mucosa, macular atrophy of the skin, hyperidrosis on the palms and soles, disturbance of the mouth opening, disturbance of swallowing, and diabetes inspidus, etc., have developed gradually.

During his hospitalization he showed severe paroxysmal hysteria, but the aplastic anemia was failed to be proved.

All symptoms of this case might be summarized due to the ectodermal disorder of the body.

Fifty-three cases, including the authors' and 14 cases of the incomplete ones, have been re-viewed.