手足掌表皮角質層增生症之一家族

Keratosis of Palms and Soles in a Family

吳立州(Lie-Jou Wu) ； 趙明志(Ming-Jyh Jau) ； 江蓉華(Jung-Hwa Chiang) ； 瞿德璐(The-Lu Chu) ； 朱夢麟(Mong-Ling Chu)

《Acta Paediatrica Sinica》 28卷1期 (1987/02) Pp. 49-52

摘要

手足掌部表皮角質增生症為一遺傳性體染色體顯性遺傳疾病，其主要病微為對稱性手足掌部表皮角層增生。大部今出生即有些微症狀，至六個月大時角化現象已非常明顯並詩續終生不褪。病灶可局限手足掌部或擴散性，忠膚與正常皮膚界限明顯。本文追綜一家族五患病成員皆犯手足掌部其中二員病灶擴及兩側肘，膝關節表層，病理切片顯出表皮各層增生，角質層尤甚。

並列摘要

Keratosis of palms and soles is a rare inherited disease transimitted as an autosomal dominant trait. It develops soon after birth and usually reaches the maximum severity by the age of 6 months. Thickening of keratin layers is diffuse but is strictly limited to these sites. The condition persists throughout life and may become accentuated by chronic friction and repeated trauma. In this report five affected members in a family are described. All affected members of this family had hyperkeratosis of the palms, soles and, in two extended to bilateral elbow and knee joint areas. Histologic examination showed hyperplasia of all layers of the skin especially the stratum corneum.

並列關鍵字

Keratosis ； autosomal dominant trait