<Clinical Studies>Cronkhite-Canada Syndrome : Epidemiological Study of 110 Cases Reported in Japan

Abstract

One hundred and ten cases of Cronkhite-Canada Syndrome (C-C-S) reported in Japan were reviewed in this epidemiologic study. Seventy-five percent of all C-C-S cases reported in the world in literature have been reported from Japan. There has been no special occupation associated with an increased incidence of C-C-S. Mental and physical stress have been confirmed as among the most important risk factors for this syndrome. Hypogeusia is the dominant initial symptom which usually is followed by diarrhea and ectodermal changes including alopecia, nail dystrophy and skin pigmentation. Gastrointestinal polyposis is closely related to the malabsorption which induced these ectodermal changes. However, there is a small number of cases in which alopecia precedes to the diarrhea in the disease course. Then, further investigation will be important in establishing the genesis of this syndrome.

Cronkhite Canada 症候群の本邦報告110例について疫学的調査を中心に検討した. 本邦では本症候群の報告は世界の報告例の75% を占めている. 本症候群の発症については職業的な特異性は認めなかった. しかし精神的及び肉体的ストレスが本症候群の発症に重要な因子であることが判明した. 味覚異常は初発症状の中, 最も重要であり, ついで, 下痢および脱毛, 爪甲異常, 皮膚色素沈着などの皮膚症状がみられた. 消化管ポリポーシスは消化管よりの吸収障害を来すものとされ, 皮膚症状の出現と密接な関係を持つものと考えられる. しかし, 下痢の発生する以前に脱毛をきたす症例も少数に認められる. 本症候群の成因はいまだに不明であるが, 本症候群のポリープには可逆性の認められることより, 炎症性ポリープが示唆され, 免疫学的機序が示唆される.