膿瘍性穿掘性頭部毛嚢周囲炎を伴つたガーゴイリズム

GARGOYLISM COMPLICATED WITH PERIFOLLICULITIS CAPITIS ABSCEDENS ET SUFFODIENS

　ガーゴイリズムはいわゆるinborn error ofmetabolismの範躊に含まれる糖質〜脂質にわたる遺伝性代謝疾患で，結合織ないし神経組織にひろく変化の及ぶ系統的疾患であり，特異な顔つき，体形を特徴とする。本症については従来小児科方面の報告が多く，皮膚科領域ではあまり知られていなかつたが，最近廻神ら^1）の論文に本症に関する詳細が紹介されているので，ここでは症例の記載を主としたい。

A 19-year-old male student has since childhood presented scaphocephaly, short neck, gargoyl appearance, macrocheilia, deformed thorax, hepatosplenomegaly, limitation of joint movement, clawhand and labyrinthine deafness. There is no clouding of cornea; intelligence is normal. Clinically, this condition is considered as Hunter's syndrome.

Urinary mucopolysaccharide excretion is markedly increased to about 600-1800 mg/day (princi-pallychondroitin sulfate B).

He has been otherwise complicated with perifolliculitis capitis abscedens et suffodiens for two years. Curettage and suture were performed, but soon after that the condition reappeared. Intra-venous injections of dextran sulfate (M. D. S.) largely reduced the urinary mucopolysaccharide excretion, but oral administrations of this drug for one year have had no effect on the urinary excretion.