6) 里吉症候群

Chracteristic Clinical Findings and Pathological Findings in the Small Intestinal Lesions of the Satoyoshi Syndrome

要旨　里吉症候群（里吉病，全身こむら返り病）は進行性筋痙攣（こむら返り），全身脱毛，下痢を3主徴とする病因不明の疾患である．下痢が高度な例ではまれに全消化管の器質的異常を認め吸収不良症候群を来すが，症例の集積も乏しくその病態は不明な点が多い．自験例を含めた報告例における小腸病変の臨床所見，病理組織学的所見の特徴は以下のごとく要約される．X線検査所見では，小腸のKerckringひだが消失し，粘膜面は大小不同の微細顆粒状隆起あるいは不整形な結節状隆起の密在から成り立ち，明らかなびらん，潰瘍は認めない．あたかも軽石状の外観（pumiceous appearance）を呈する．内視鏡検査所見では，粘膜面はまだら発赤調で，散布性白斑を伴う微細顆粒状の粘膜変化や腫大した絨毛の集簇から形成されたポリープ状の隆起を認める．高度の消化器症状を呈した症例の剖検病理組織所見では食道を除く全消化管にgastroenterocolitis cystica polyposa（GCP）の像を呈する．

　Satoyoshi is a disease of unknown etiology characterized by three principal manifestations: progressive muscle spasms (cramps), generalized alopecia, and diarrhea. In rare cases in which the diarrhea is severe organic abnormalities are observed throughout the entire digestive tract, and they give rise to a malabsorption syndrome, but few cases have been accumulated, and many aspects of the pathology remain unknown. The characteristic clinical findings and histopathological findings in the small intestine lesions in the cases that have been reported, including our own, can be summarized as follows. The radiographic findings show loss of Kerckring's folds in the small intestine, and the mucosal surface consists of densely arrayed fine granular protrusions of various size or irregularly shaped nodular protrusions, but no clear erosions or ulcers are seen. It has a pumiceous appearance. Endoscopically, the mucosal surface has a mottled red appearance, and fine granular mucosal change with scattered white patches, or polypoid protrusions formed by clusters of enlarged villi are seen. The histopathological findings at autopsy in the cases that have exhibited severe digestive system manifestations have shown a picture of gastroenterocolitis cystica polyposa (GCP) throughout the digestive tract except in the esophagus.