長期経過例からみた腸型Behçet病と単純性潰瘍の病態

Clinical Course of Intestinal Behçet's Disease and Simple Ulcer

　単純性潰瘍6例，腸型Behçet病7例(完全型1例，不全型6例)に対し平均5.1年(1～13年)の経過観察を行った．初回，単純性潰瘍と診断された2例(16歳，27歳)で，腸管病変出現とほぼ同時に現れた口腔内アフタに加え，両者ともほぼ1年後に皮膚症状が出現し，不全型Behçet病の基準を満たした．最終診断は単純性潰瘍4例，腸型Behçet病9例で，平均発症年齢はそれぞれ38.2歳，28.4歳，腸管病変診断時年齢は44.0歳，34.6歳といずれも単純性潰瘍が約10歳高齢であった．先行する感冒様症状や経過中の難治性再発性扁桃炎の頻度は両疾患の間に差はみられなかった．単純性潰瘍は，高齢発症し，症状が揃いにくい腸型Behçet病の一亜型として捉えられる可能性が考えられた．腸管病変の内視鏡所見の特徴によって対象症例は4つに分類された．①回盲弁または近傍に単発する2cm以上の大きな辺縁明瞭な打ち抜き様潰瘍，②回盲部に多発する小型円形潰瘍，③回盲弁以外の，大腸の広い範囲にわたって多発する打ち抜き様潰瘍，④回盲部，主に終末回腸に多発するびらん．すべての症例で複数の治療が行われており，回盲弁の変形を伴う①，②の症例では腸管病変の改善はほとんどみられなかったがC回盲弁に病変，変形を認めなかった③，④の症例では保存的治療に反応し軽快した．

　We studied the clinical course and the intestinal lesions in 9 patients with intestinal Behçet's disease and in 4 patients with simple ulcer. At the first examination, two cases with intestinal Behçet's disease showed only intestinal lesions and oral ulcers, so they were diagnosed as simple ulcer. However about one year later, skin lesions appeared in both cases and their diagnosis was corrected to intestinal Behçet's disease, incomplete type. The average age of patients at onset and diagnosis of simple ulcer was about 10 years older than the average age of patients at the onset of intestial Behçet's disease. No other significant differences were found in the clinical course or characteristics of intestinal lesions in both diseases. So, the disease entity of simple ulcer may be a sub type of Behçet's disease, which begins at an older age and is not usually accompanied with other symptoms.

　Our cases were divided into four groups by the characteristics of the intestinal lesions ; ① undermining large ulcer on the ileocecal (IC) valve ② multiple small ulcers on and around the IC valve ③ multiple undermining ulcers in many areas of the colon, without any lesions on the IC-valve ④ multiple erosions mainly located in the terminal ileum. With medical treatment, intestinal lesions in the cases characterised by ③ or ④, without deformity of the IC-valve, showed improvement, but lesions of type ① and ② with deformed opened IC-valve did not improved.