Ichthyosis Hystrix of Curth-Macklin

Curth-Macklin氏棘狀魚鱗癬

10.29784/DS.200409.0005

楊弘旭(Hung-Hsu Yang)；吳南霖(Nan-Lin Wu)；林揚志(Yang-Chih Lin)；吳育弘(Yu-Hung Wu)

Ichthyosis hystrix ； Epidermolytic hyperkeratosis

中華皮膚科醫學雜誌

22卷3期（2004 / 09 / 01）

239 - 242

英文

Curth-Macklin氏棘狀魚鱗癬是一種極少見的角化異常症。臨床上可見到不等程度疣狀到棘狀的過度角化。病理特徵為表皮鬆解性過度角化及雙核表皮細胞。電子顯微鏡下呈現圍繞細胞核的特有的殼狀構造，可作為診斷依據。我們報告一Curth-Macklin氏棘狀魚鱗癬病例，並針對臨床，病理及基因做一文獻的回顧。

Ichthyosis hystrix of Curth-Macklin (IHCM) is an extremely rare keratinization disorder, with verrucous to hystrix-like hyperkeratosis plaques of varying extent. The diagnosis is based on a distinctive pattern of epidermolytic hyperkeratosis and the presence of binucleate cells under light microscopy, as well as unique electron microscopic findings of continuous perinuclear tonofibril shells in the suprabasal keratinocytes. We present the clinical manifestation and light and electron microscopic findings in a 23-year old male patient with IHCM.