Melanoses neurocutaneesの1例

MELANOSES NEUROCUTANEES

　1949年Touraine^1）は皮膚と軟脳膜に共通の色素性病変を有する疾患をMélanoses neurocu-tanéesと称し，その綜説を発表したが，その報告例はまだ数10例に過ぎず，本邦においても20例に満たない稀有な疾患である。

　最近，著者は生前にMélanoses neurocu-tanéesを疑い4年後に中枢神経系の悪性黒色腫により死亡し，剖検によつて本症を確定診断することができた症例を経験したので，本邦報告例と比較考察し以下に報告する。

Nineteen cases of this rare disease have been reported in Japan. A 19-year-old man with disseminated nevo-cellular nevi over almost the entire body surface, a trouser-shaped giant nevus and a giant lipoma on the lower half of the body was reported.

The clinical diagnosis was given from the skin manifestations and the abnormal EEG findings at the age of 16. He died of a malignant melanoma of the central nervous system which was assumed to be transformed from the melanose of the pia mater encephali.

The diagnosis was confirmed by autopsy.

The possibility of giving diagnosis of this disease while alive was discussed.