21年間の経過観察中に再発を繰り返した腸型Behçet病の1例

Repeated Recurrence of Intestinal Behçet's Discase Observed over a 21-year Follow-up Period, Report of a Case

　患者は36歳，男性．Behçet病の主症状4項目のうち，眼症状がなく，陰部潰瘍，口腔内アフタ，皮膚症状のある，不全型Behçet病だった．画像検査で回盲弁上に境界明瞭な，深く，大きな潰瘍を認め，腸型Behçet病(本疾患)と診断された．保存的な治療に反応ないため，回盲部切除術を施行した．5年後の画像検査で吻合部に瘻孔形成を認め，その後保存的治療を開始したが効果なく，瘻孔は増悪傾向だった．症状も増悪し，1回目の手術12年後に吻合部および一塊となった回腸の切除術を行った．再手術後早期に認めた数か所の小さな潰瘍性病変は，経時的に消褪，再発を繰り返していたが，5年後に腸管の漿膜側への炎症の波及を示唆する所見が強くなり，潰瘍は深く，大きく典型的な本疾患の形態となった．2回目の手術から7年後に潰瘍は皮膚瘻を形成したため，再々手術となった．21年間の経過観察中に，3回の腸切除が行われ，小病変から本疾患に典型的な下掘れ潰瘍の形態を呈する経過を観察できた症例を経験したので報告した．

　A 36-year-old male patient complained of abdominal pain and aphthoid ulcers of the oral cavity, genital ulcers and acne-like eruptions but no eye symptoms. We diagnosed incomplete Behçet's disease. Barium enema examination showed a deep, undermining, oval ulcer on the ileocecal valve. Being diagnosed with intestinal Behçet's disease, the patient was treated with an elemental diet, but this therapy was not effective, We conservatively performed ileocecal resection without reaction. Barium enema five years after the first operation revealed fistula in the anastomotic site, and we started conservative treatment, but it was ineffective and the fistula was aggravated as the course of treatment continued. The symptoms improved, but 2 years after the operation, surgery was performed to remove a lump and the ileum and the anastomotic site was re-operatetion. The small ulcers near the anastomotic site showed further recurrence with time in the same area. 5 years after reoperation, findings outside the bowel became clearer, and an ulcer lesion was typical of intestinal Behçet's disease. Because a skin stoma was formed, we operated again seven years after the 2nd operation. In a follow-up period of 21 years, enterectomy was carried out three times. We reported it because it was a case in which we were able to observe the course which course of the disease which presented configuration of a typical, deep, undermining, oval ulcer which started as a small lesion.