Introduction 1 2 3 4 5 7 Materials and Methods 1 1 2 2 Table 1 The Mason–Barnes Criteria Major Symptoms Minor Symptoms Buccal ulcerations Gastrointestinal lesions Genital ulcerations Thrombophlebitis Ocular lesions Cardiovascular lesions Skin lesions Arthritis Neurologic lesions Family history Three major or two major and two minor criteria are required to establish the diagnosis of Behçet’s disease Table 2 n Case No. Age (years) Sex Oral Ulcer Genital Ulcer GI S & S Ocular Signs Skin Lesion Pathergic Reaction Arthritis or Arthalgia 1 38 M + + + − − + + 2 45 M + + + + +  + 3 26 F + − + + −  − 4 47 M + + + + +  − 5 28 F + − + + − + + 6 36 F + + + + −  − a 22 M + + + − − +  8 42 M + + + + −   9 22 M + + + − + + + 10 28 F + + + + −  − 11 65 M + + + − +  − a 23 M + + + − + − − 13 32 F + − + + +  − 14 24 M + + + + − + + 15 34 M + + + − −   16 41 F + + + − +   b 38 M + + + + + + − 18 33 M + + + − + − − 19 25 M + + + − + + + 20 48 F + + + + −   21 29 M + + + − + − − 22 50 F + + + + −  + Plus signs mean that the feature is present; minus signs mean that the feature is not present. CTH = Cardinal Tien Hospital, TSGH = Tri-Service General Hospital, S & S = symptoms and signs a b In 13 of these 22 cases, the diagnosis was confirmed at surgical resection for multiple perforations. Nine of the 22 cases had Behçet’s disease with intestinal involvement, which was confirmed preoperatively, six were confirmed by endoscopic examination; two by radiological examination; and one patient had gastrointestinal symptoms of intermittent abdominal pain, diarrhea, and nausea. Results Patient Characteristics There were 14 men and 8 women in the 22 cases investigated. The ages of the patients with perforated intestinal Behçet’s disease ranged from 22 to 65 years, with a mean age of 35.3 years. The age at onset of symptoms of Behçet’s disease varied from 18 to 64 years on diagnosis, with a mean age of 33.1 years. 2 1 2 3 4 Figure 1 Buccal ulcer.  Figure 2 Penile ulcer.  Figure 3 Nodular cutaneous lesion on the back.  Figure 4 Vasculitis characterized by lymphocytic and plasmacytic infiltration of perivascular tissue (hematoxylin and eosin; 10 × 40).  3 Table 3 Laboratory Data Case No. Immunoglobulins (mg/dl) Serum Complement (mg/dl) Globulin (%) IgG IgA IgM C′3 C′4 1 1,976 375 250 145 38 13.8 23.8 2 1,726 245 174 92 40 12.0 10.8 4 2,150 400 240 110 45 14.2 24.6 5 1,500 590 300 38 25 10.5 18.0 a 740 185 60 90 38 7.8 14.3 8 1,180 195 140 59 32 6.6 12.2 9 2,270 464 262 127 46 14.0 16.2 11 1,850 380 250 190 50 12.5 23.5 a 1,300 320 235 88 39 9.6 15.0 14 2,350 490 295 180 48 13.3 25.0 16 680 98 56 150 35 13.0 21.8 b 1,650 475 280 76 34 9.4 12.5 18 1,800 290 150 105 45 13.8 23.2 19 2,418 581 209 166 40 14.4 28.0 21 1,880 330 250 180 35 10.5 20.0 22 1,985 386 228 168 38 13.8 24.2 Normal range 950–2,110 170–410 54–262 47–191 27–52 4.8–12.1 8.8–22.8 a b 5 4 Figure 5 Surgical specimen of ileocecal region showing multiple penetrating ulcers.  Table 4 Operative Findings and Operation Performed in 22 Perforated Intestinal Behçet’s Disease Patients Case No. Location of Perforated Ulcers No. of Perforations Oral/genital Ulcer Operation Performed 1 Terminal ileum 4 +/+ Partial resection of the ileum 2 Terminal ileum 6 +/+ Partial resection of the ileum 3 Ileocecal region and ascending colon 10 +/− Right hemicolectomy and ileocecal resection 4 Ileocecal region and ascending colon 16 +/+ Right hemicolectomy and ileocecal resection 5 Cecum and ascending colon 5 +/− Ileocecal resection 6 Terminal ileum 5 +/+ Partial resection of the ileum a Terminal ileum 4 +/+ Partial resection of the ileum 8 Cecum and ascending colon 9 +/+ Right hemicolectomy and ilececal resection 9 Terminal ileum 8 +/+ Partial resection of the ileum 10 Ileocecal region and ascending colon 11 +/+ Right hemicolectomy and ileocecal resection 11 Ileocecal region and ascending colon 10 +/+ Right hemicolectomy and ileocecal resection a Terminal ileum 5 +/+ Partial resection of the ileum 13 Terminal ileum 7 +/− Partial resection of the ileum 14 Ileocecal region and ascending colon 11 +/+ Right hemicolectomy and ileocecal resection 15 Ileocecal region and ascending colon 5 +/+ Right hemicolectomy and ileocecal resection 16 Ileocecal region and ascending colon 13 +/+ Right hemicolectomy and ileocecal resection b Cecum and ascending colon 4 +/+ Ileocecal resection 18 Ileocecal region and ascending colon 7 +/+ Right hemicolectomy and ileocecal resection 19 Ileocecal region and ascending colon 9 +/+ Right hemicolectomy and ileocecal resection 20 Cecum and ascending colon 6 +/+ Ileocecal resection 21 Terminal ileum 4 +/+ Partial resection of the ileum 22 Ileocecal region and ascending colon 12 +/+ Right hemicolectomy and ileocecal resection a b Operative Treatment and Outcome All 22 perforated intestinal Behçet’s disease cases were confirmed at operation, with nine of them correctly diagnosed preoperatively. Surgical resection of the perforated intestinal ulcers was done in all cases, with right hemicolectomy and ileocecal resection in 11 cases, partial ileum resection in 8 cases, and ileocecal resection in 3 cases. No reperforation occurred in the group of patients who underwent right hemicolectomy and ileocecal resection. However, two reperforations ocurred in patients who underwent partial ileum resection alone and one in the ileocecal resection group. 6 Figure 6 Chronic inflammatory response and perivascular infiltration (hematoxylin and eosin; 10 × 10).  After operation on these 22 patients with Behçet’s disease and intestinal perforation, four patients died during the postoperative course due to septic shock, which was present prior to the surgical intervention; three died from complications of hypertension and diabetes mellitus; and three were lost to follow-up. Thus, only 12 patients are still under observation, without evidence of gastrointestinal complications up to this date. The remaining 60 cases of intestinal Behçet’s disease, without perforations, are still under surveillance. Discussion 5 6 2 3 4 3 2 4 8 9 2 5 6 8 10 30 3 2 4 11 12 12 13 1 14 15 16 16 17 1 18 19 5 20 3 4 21 2 22 24 25 29 3 4 8 30 31 2 7 30 5 3 4 Conclusion 4 31