Intestinal Behçet Syndromeについて

INTESTINAL BEHÇET SYNDROME

　Behçet病（Behçet，1937^1,2））は粘膜・皮膚・眼の3大病変のほか関節，消化管系，血管系，中枢神経系など全身的に多種多彩な炎症々状を呈することが諸家によつて報告されている。塚田ら^3）の総説によると粘膜・皮膚・眼以外の諸臓器における症状の発生頻度は報告者により若干の差はあるが，さほどまれなものではない（表1）。

　これらの病変のうち生命の予後に重大な影響を与えるものは中枢神経系，血管系ならびに消化管系の病変であり，それぞれneuro-Behçet syndrome^4〜6），cardio-Behçet^7），vasculo-Behçet^8），あるいはangio-Behçet syndrome^9,10），intestinal Behçet syndrome^11）と呼ばれている。

A 47-year-old woman of Behçet's disease without the eye symptom who died of the repeated profuse bleeding due to ulcers and tumors at the end of cecum and the colon ascendens after 8 years' course.

Histopathologic pictures from the bowel and external genitalia were the same, showing hyalinous degeneration, fibrin deposition, and thickening of the blood vessel wall, thrombus formation, perivascular dense infiltration of lymphocytes and neutrophile leucocytes.

Significant differences of the results of cutaneous reactions with 6 kinds of bacterial antigens were observed between the patients of Behçet's disease and normal controls. Common positive reactions were produced by streptococcus viridans and hemolyticus, while the reaction by pneumococcus was negative in the incomplete type of the disease.

These findings suggested that pathognomonic changes of this disease might be an allergic vasculitis.

Laboratory tests revealed increased γA, γM, and γG fractions of patients' serum.