Cronkhite-Canada症候群の1剖検例

An Autopsy Case of Cronkhite-Canada's Syndrome

　遺伝歴がなく，脱毛・皮膚色素沈着・爪甲脱落・低蛋白血症を伴うびまん性消化管polyposisは，1955年初めてCronkhite and Canada¹⁾によって報告され，以来この稀有な疾患はCronkhite-Canada症候群と呼ばれている．最近われわれはこの症候群にきわめて類似した1例を経験し，剖検する機会を得たので報告する．

症例

　患　者：62歳男，職工．

　既往歴：50歳時に高血圧・心筋障害で約1カ月間入院した以外に特記すべきものなし．

　家族歴：両親・長兄が脳卒中で死亡した以外，特記すべきものなし．

　An autopsy case of Cronkhite-Canada's syndrome is reported. The patient, 62 years old male, was admitted with the complaints of diarrhea and poor appetite, later developing loss of nails, alopecia, and skin pigmentation. The family history was non-contributory. Laboratory investigation revealed marked hypoproteinemia (total serum protein was 2.1 g/dl in late stage), hypocalcemia, low level of serum iron and copper, and hypoacidity of the gastric juice. All the function tests of the liver, kidney and endocrine revealed normal results. Roentgenographic and endoscopic examinations showed diffuse polyposis in the stomach, colon and rectum. Gastroendoscopic features were very dramatic. The gastric antrum was covered with innumerable polypoid lesions which had not been seen one month before. The patient died from ileus 22 months after the onset of symptoms.

　The microscopic picture not only of polyps but also of the other parts of the gastrointestinal mucosa was highly uniform and characterized by marked proliferation of mucus-secreting glands without evidence of malignant change.

　Cronkhite-Canada's syndrome characterized by diffuse gastrointestinal polyposis, alopecia, nail dystrophy, and skin pigmentation, is extremely rare, and only 23 cases have been described until now. Thus, the present case seems to be the twenty-fourth. Clinical and pathological data of those 24 cases are reviewed as follows. Fifteen are male and nine are female. The onset of illness was after middle age and the initial symptom was diarrhea in most patients. Polyps are invariably present in the stomach and colon. In 11 autopsy cases, involvement of the jejunum is mentioned only in two cases. Malignant transformation of the polyps is reported in two cases. The illness ended in death in 13 cases, and 10 survived.