網状肢端色素沈着症（北村）症例追加

THREE CASES OF ACROPIGMENTATIO RETICULARIS KITAMURA

I．はじめに

　本症は昭和18年北村・赤松^1）が，「一種の対側性肢端色素異常症」なる病名で，18才男子の症例を報告したのが最初で，その後昭和28年^2）その特異な臨床像および組織像よりAcropigmentatio　reticularis kitamura （網状肢端色素沈着症・北村）なる名称が提唱され，今日迄既に多数の症例の集績をみている。我々は最近3代28名中，9名の本症をみる家系で，2代3名の本症を経験したので報告する。

Three cases of acropigmentatio reticularis in one family were reported. Nine of 28 members in 3 generations of the family tree had this disease.

Case 1 : A 19-year-old girl noted slightly depressed small fleckles on the back of handssince the age of 10, which increased in number gradually, especially in summer. On the palms and soles there were no fleckles, but small spots without finger-print were proved.

Histologic specimen showed that one macroscopic fleckle was composed of many small pigmented areas, the malpighian layer of which showed various degree of atrophy with increa-sed number of rete ridges and Dopa-positive melanocytcs.

Case 2 : A 13-year-old girl, sister of case 1, noted the same eruption since the age of 10. The degree of her skin manifestions was milder than case 1.

Case 3 : A 50-year-old man, father of case 1 and 2, had the same kind of skin disorder since childhood. It occupied almost entire body surface.

In all 3 cases punctate disappearence of finger-print was proved. Its pathognomonic change might not only be pigment disorder, but also atrophy of the epidermis.