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Clinico-pathological Studies on the Intestinal Lesions in Behçet's Disease-with special reference to Entero-Behçet's syndrome T. Shimizu 1 , T. Ogino 1 12nd Department of Internal Medicine, Teikyo University School of Medicine pp.1593-1600
Published Date 1975/12/25
DOI https://doi.org/10.11477/mf.1403112113
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 It is well known that aphthous stomatitis, erythema nodosum, pyoderma, arthritis and even eye lesions do occur in ulcerative colitis, as well as in regional enteritis. Whether these are integral part of the host response to an etiologic agent in parallel with the intestinal lesions, or whether they are secondary to the intestinal, remain unproved.

 On the other hand, the sympotomes of Behçet's disease are: aphthous and ulcerative changes both in the oral mucosa (99%) and in the genital area (69%), ocular disturbances (uveitis) (90%) usually leading to blindness, polymorphous skin manifestation (84%) (erythema nodosum, etc.) and arthritis (52%). In addition, there are intestinal lesions, the symptomes of which are abdominal pain, diarrhea, constipation and melena, etc., (23.3%). Then, what is the difference of the intestinal lesions among Behçet disease, ulcerative colitis and regional enteritis (Crohn's disease)? These diseases may be called “muco-cutaneous-intestinal syndromes”.

 We have experienced more than 216 cases (132 males and 84 females) of Behçet's disease, and investigated the lesion of the small and large bowel by roentogenological, endoscopical and histological methods.

 The results;

 (1) Radiological study on the small bowel, (58 cases). The major X-ray findings were: 1) coarsening of mucosal folds and appearance of hypersecretion, flocculation, scattering, segmentation and frag-mentation, (23.3%). 2) abnormal dilatation of the intestinal loops, and hypotonus, (18.6%). 3) hyperkinetic segmentation, (23.3%). 4) gas distension, so called “Aufstand”, “Rahmenbeschlage”, (32.6%). 5) normal, (25.6%).

 (2) Radiological study on the large bowel, (38 cases). We found erosions and ulcers, (10.5%).

 (3) Colonoscopy showed an edematous and congested easily bleeding mucosa and ulcers with sharp margin, especially in ileocecal region, including Bauhin's valve, (10 cases).

 (4) Histopathological findings of 30 cases (including operation material 21 cases, autopsy 7 cases, and biopsy 2 cases) were as follows;

 1) In spite of the superficial necrosis of inflammatory granulation tissue and the penetration of the ulcers even into the subserosa, scant of if any collection of neutrophil leucocytes was seen.

 2) The defect was replaced with the granulation tissue accompanied with the infiltration of lymphocytes, plasma cells, a few eosinophils and a few reticulohistiocytes, but with relative lack of fibrosis. In almost all cases lymph follicle formation was a striking feature. Focal collections of lymphocytes often with prominent follicle formation were scattered through all layers of the intestinal wall.

 3) There existed a marked evidence of vasculitis of diverse morphology in the vessels (precapillaries, capillaries, arterioles and venules), even in the distant places from ulcers. This consisted of swelling and proliferation of endothelium, characteristic degenerative histolysis and reactive hyperplasia of media muscle, bizarre changes of elastic fibers, sometimes fibrinoid necrosis of all the wall elements, lymphocytic infiltration of muscle layer, fresh or organized thrombi.

 4) In some cases the submucosal layer was edematous and the fibers of muscularis propria were separated by edematous exudate becoming stretched and thinned.

 5) Nothing was characteristic with intramural nervous apparatus.

 (5) We named the type of Behçet's disease as “Entero-Behçet's syndrome” in which the symptoms occured by the intestinal lesions form its main symptoms.


Copyright © 1975, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1219 印刷版ISSN 0536-2180 医学書院

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