全消化管にポリポーシスを認めたCowden病の1例

Cowden's Disease, Report of a Case

要旨　患者は43歳，女性．胃集検にて胃のポリポーシスを指摘され来院．消化管検査にて食道から大腸までの全消化管にポリポーシスを認めた．病理組織学的に検索された病変はいずれも過形成性変化を主体としたものであった．顔面，四肢の角化性小丘疹，口腔粘膜の乳頭腫症などの特徴的所見から，典型的なCowden病と診断した．本症例には腺腫様甲状腺腫，胆囊ポリープ，肝血管腫，脂肪腫（四肢）も合併していた．本例では特にCowden病の小腸病変を明瞭に描出できたので報告する．

　Cowden's disease is a rare disease characterized by multiple hamartoma syndrome. To our knowledge, only 30 cases have been previously reported in Japan.

　We reported a case of Cowden's disease in a 43-year-old woman accompanied by polyposis of her entire digestive tract. Small keratotic papule on the face and extremities, papillomatous lesions in the oral cavity, adenomatous goiter, gallbladder polyp, hepatic hemangioma and subcutaneous lipoma of the left foot were also observed.

　In this case, multiple small polyps in the small intestine were clearly demonstrated on double contrast barium study.