痙攣発作を伴つた尋常性魚鱗癬の1例

A CASE OF ICHTHYOSIS VULGARIS ASSOCIATED WITH CONVULSIVE SEIZURE

I．緒言

　組織発生の源を一にする皮膚と中枢神経系はそれぞれの障害を合併することが知られている。たとえば結節硬化，Sturge-Weber病，Reckling—hausen病等は皮膚疾患とともに痙攣発作，精神薄弱等が合併する。

　Yakovlev　＆　Guthrie^17）は上記3疾患をcon—gental neurocutaneous syndrornesと呼び外胚葉に由来する器官—神経系，眼球網膜および皮膚に選択的に起こつたcongenital maldevelo—pmentの結果であつて臨床的にも病理学的にもcongenital ectodermosesというひとつのグループに属すると考えた。またこれら3疾患の相違は障害の起こるembryogenesisの時期，中胚葉要素の関与の程度により，さらに精神薄弱とてんかんの合併は原始外胚葉（primitive ectoderm）の奇形によつて起こつた神経機能のdefective　integrationの結果と考えた。

A case of ichthyosis vulgaris associated with convulsive seizure was reported. Man aged eleven years. His skin revealed dysker-atosis like a goose-skin, and dermatological diagnosis was typical ichthyosis vulgaris. From his age of five months he had convulsive seizures which diminished after his age of five years. But seizures reappeared before several months. The seizure was generalized clonic-tonic convulsion. Except for the patient, in the family who suffered from ichthyosis vul-garis, no one had convulsive seizures. As for the skin disease, the mode of inheritance was dominant. Electroencephalograms showed irregular trace with high voltage slow wave, but reduced considerably after pneumoence-phalography. His mother's electroencephalogr-ams showed normal. Rud and others reported kryptorchism as the complication of ichthyosis vulgaris, but endocrinological abnormalities including this complication were not observed in this case. As for the association with ichthyosis vulgaris and convulsive seizure, it may be assumed that the gene of ichthyo-sis vulgaris showed polymeric manifestation. Therefore, it may be considered that there exists uncomplete type of Rud syndrome as this case. From the embryological standpoint, skin and central nervous system develop from the same ectoderm, therefore, the syndrome of ichthyosis vulgaris associated with convu-lsive seizures may be included a group of congenital ectodermoses (Yakovlev & Guthrie) or congenital neuro-ectodermal clysplasis (Van Bogaert).