| 特集名 | 消化器疾患と皮膚病変 | |
|---|---|---|
| 題名 | Cronkhite-Canada症候群と皮膚病変 | |
| 発刊年月 | 1999年 12月 | |
| 著者 | 川口 淳 | 防衛医科大学校第二内科 |
| 著者 | 永尾 重昭 | 防衛医科大学校第二内科 |
| 【 要旨 】 | Cronkhite-Canada症候群は原因不明の消化管全体にわたる非遺伝性ポリポージスである．日本人の報告例が全世界の約3/4を占める．初発症状は脱毛，爪甲萎縮，味覚異常，皮膚色素沈着などの皮膚病変と下痢，体重減少，腹痛，浮腫，テタニーなどがみられ蛋白漏出性胃腸症をきたし従来は予後不良とされていた．近年，副腎皮質ステロイド，中心静脈栄養，経腸栄養，抗プラスミン製剤などにより病状の改善がみられる．病状の改善とともに消化管ポリープは縮小，消退し，それに伴い皮膚病変，栄養状態も改善する．癌合併はまれとされていたがポリープの癌化に注意する必要がある． 吸収不良症候群は，糖質，脂質，蛋白質，ビタミン，ミネラル，水，電解質などの各種栄養素の消化・吸収が障害され，各種栄養素の欠乏に基づく種々の臨床症状を呈する疾患・病態を包括した総称である．吸収不良症候群に伴う皮膚病変は消化吸収障害の結果惹起される各種栄養素の欠乏に基づくと考えられる． |
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| Theme | Gastrointestinal Tract Disease and Skin Manifestation | |
|---|---|---|
| Title | Cronkhite-Canada Syndrome and Cutaneous Manifestations | |
| Author | Atsushi Kawaguchi | 2nd Department of Internal Medicine, National Defense Medical College, School of Medicine |
| Author | Shigeaki Nagao | 2nd Department of Internal Medicine, National Defense Medical College, School of Medicine |
| [ Summary ] | Cronkhite-Canada syndrome is an unknown etiologic generalized gastrointestinal polyposis, with pathologically diagnosed juvenile type polyps. About seventy five cases have been reported in Japan. Clinical manifestations are cutaneous ones ; diffuse alopecia, nail changes (i.e. onychomadesis), onychodystrophy etc., hypogeusia with pigmentation, malabsorption syndrome due to polyposis (i.e. diarrhea, weight loss, anorexia, abdominal pain, edema, and tetanii etc). There is no evidence of genetic transmission. Recently, various medical and surgical measures have been employed in the management of this disease including corticosteroids, dietary supplementation with oral and/or intravenous fluids, electrolytes, vitamins, minerals, amino acids, albumin, lipids, anti-plasmin drugs. These treatments have been achieved good results. Polyps in the alimentary tract as well as cutaneous lesions are diminished or eliminated through the therapy. Other clinical manifestations and nutriotional condition may also be improved. It used to be said that cancer rarely accompanied this disease, but recently associated cancers have been reported and must be given attention. In cases of malabsorption syndrome various symptoms are caused due to maldigestion and/or malabsorption of glucose, lipids, proteins, vitamins, minerals, electrolytes, water, etc.. It is assumed that the cutaneous lesions seen in this syndrome are due to a deficiency in various nutritional elements. |
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