Incontinentia pigmenti知見補遺

NEW CONCEPT ON INCONTINENTIA PIGMENTI

I．Incontinentia pigmenti （I．p．）の発症過程

　I．p．の初発期に発赤腫脹・苔癬様丘疹あるいは帯状疱疹様小水疱形成の病変を前駆することはBloch-Sulzberger^1）以来指摘された所で，生下時あるいは生後間もなく発現し，しかも数年で色斑の消褪する経過とともに主要な特徴Prototypeである。しかし成年期まで該色斑の一部を稽留する異例もSulzberger-Fraser-Hutner^2）始め数例の報告があり，私^3）も26歳女子で経験してI．p．continuaと称うべき亜型と考えている。

　さらに最近私はi）9歳の少女で2年前麻疹様で初発せる例ii）25歳の女子で1カ月前より左側の胸部ならびに上肢屈側に紅斑小丘疹を展開せる例iii）23歳の女子で半年前に右膝膕に苔癬様皮疹を発生し下肢後面において漸次上下に進展した例がそれぞれその後I．p．に一致する色斑に変転せる患者を経験し，その組織学的所見もI．p．典型例と合致したもので，正にI．p．　tardaとも称えられるべきものと思推されるが，さらに症状ならびに経過の酷似するLichen striatusについて演繹考究したい。

There exist several atypical forms of incontinentia pigmenti. In incontinentia pigmenti continua pigmented macules persist till adulthood. Incontinentia pigmenti achromians shows depigmented area as a negative picture of typical incontinentia pigmenti.

Incontinentia pigmenti tarda starts after childhood. The author presented one case with pigmented macule which resembled typical form in the course of lichen striatus and disappeared gradually. This atypical form seems to be easy to occur in Mongolians who have many melanin disorders of the skin.

Histologic specimen from holy-leaf-shaped pigmented macule showed melanin granules in the macrophages around the capillaries in the upper dermis, but there was no free melanin in the dermis. Electronmicrogram revealed active phagocytosis of melanin granules into the macrophages in the papillary layer, which extended pseudopod-like process just beneath the basal layer of the epidermis.

These findings suggest that a kind of congenital or aquired pathologic process under the influence of physiological function of vasomortor nervous system might act on the basal cell layer of the epidermis which may make pigment incontinent and mobilization of histiocyte.

Its predilection in female may be due to some disturbance on X-chromosome.