発生異常に基づく神経・皮膚症候群

Nurocutaneous Syndromes

I．はじめに

　皮膚と中枢神経とを同時に侵す疾患は少なくない。これに神経・皮膚奇形および同奇形性疾患（neurocutane Miβbildungen und Miβbildungs-krankheiten），新陳代謝疾患にもとづく神経・皮膚症候群（neurocutane Syndrome auf dem Boden von Stoffwechselleiden），急性および慢性伝染病における神経・皮膚症候群（neurocutane Syndrome bei akuten und chronischen Infektionskrankheiten），アレルギー性神経皮膚症候群（neurocutane Erscheinungen allergischer Natur）などがある^1）。またその他にも，たとえば全身性紅斑性狼瘡，いわゆる細網肉腫症や転移性悪性黒色腫などに見られる脳血管障害にもとづく中枢神経症状など，皮膚科医が遭遇するこの種の症候群は少なくない。このところではそれらのうちこの特集号の「奇形性神経疾患」という範疇に属するものについて述べることにする。それらのうちでもつとも重要なものは母斑症およびその類症である。まずそれらについて述べ，次に母斑症とは異なる先天性疾患で皮膚と神経系統とに同時に病変が生ずることが問題となるようなものについて述べたい。

There are numerous diseases in which lesions are revealed in the skin and in the central nervous system concomitantly. Their pathogeneses are various. The conditions to be discussed here are those caused by the disturbances of ontogenesis. The most important disorders among the neurocutaneous syndromes of dysontogenetic genesis are thephacomatoses, where nervus-like lesions occur not only in the skin but also in the central nervous systems and other organs. The phacomatoses have by no means common pathogenesis. The Bourneville-Pringle's phacomatosis and the von Hippel-Lindau's phacomatosis seem to be caused by the disturbance of the development of the mesenchym. The Recklinghausen's phacomatosis and Méanose neurocutandé of Touraine seem to be caused by the disturbance of the devAopment of the neural crest which gives rise to the melanocytes and Schwannian cells. The Sturge-Weber's syndromeis allied to phacomatosis. An example of neurocutaneous syndrome caused by congenital biochemical defect is the phenylketonuria. The pseudoxanthoma elast;cum or Groenblad-Strandberg's syndrome is a systemic disorder consisting in a peculiar degeneration of the elastic tissue. Its most conspicuous lesions are those in the skin and ocular foundus. However, cardio-vascular and brain lesions may also be found. The xeroderrna pigmentosum may be accompanied by the shortage of the intelligence.