Kanai M, Kondoh S, Kuriki H, Mukaiyama H, Mori K, Tanno T
Department of Surgery, Daidoh Hospital, Nagoya, Japan.
Nihon Geka Gakkai Zasshi. 1988 Jul;89(7):1127-31.
A 47-year old male complaining of severe abdominal pain associated with distention was admitted to our department on August 5, 1986. His first admission to our hospital was 18 days previously for leg pain and fever. He underwent emergency operation with a preoperative diagnosis of acute peritonitis due to perforation of gastric ulcer. Operative findings showed one perforation of the stomach and two of the ileum. Distal gastrectomy, enterectomy and peritoneal drainage were carried out. Resected specimen revealed six ulcers, two of them in the stomach, four in the ileum. Microscopic examination disclosed intimal proliferations of small arteries in the mucosal layer. The vessels near the ulcers were most severely involved but the same changes were also found in the subserous layer and mesentery. It was suggested that the multiple ulcers were secondary to vascular lesions identical to the gastrointestinal lesion of Degos' disease. Postoperative examinations revealed one ulcer in the jejunum and another in the descending colon. Ten months after operation he lives with no complaint on the gastrointestinal tract. Only 80 cases of Degos' disease have been reported in the western countries and 10 cases in Japan. In those atypical cases of Degos' disease without papulosis were only Manuel's and ours.
一名47岁男性，因严重腹痛伴腹胀于1986年8月5日入院。他18天前因腿痛和发热首次入住我院。术前诊断为胃溃疡穿孔所致急性腹膜炎，遂行急诊手术。手术发现胃有1处穿孔，回肠有2处穿孔。行远端胃切除术、肠切除术及腹腔引流术。切除标本显示有6个溃疡，其中2个在胃，4个在回肠。显微镜检查发现黏膜层小动脉内膜增生。溃疡附近的血管受累最严重，但浆膜下层和肠系膜也有同样改变。提示多发性溃疡继发于与迪戈斯病胃肠道病变相同的血管病变。术后检查发现空肠有1个溃疡，降结肠有另1个溃疡。术后10个月，他无胃肠道不适症状。西方国家仅报道了80例迪戈斯病病例，日本有10例。无丘疹的迪戈斯病非典型病例只有曼努埃尔的病例和我们的病例。
