Confusing untypical intestinal Behcet's disease: Skip ulcers with severe lower gastrointestinal hemorrhage

Abstract

Behcet's disease (BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileo-colonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.

Keywords: Hemorrhage; Intestinal Behcet’s disease; Skip ulcers.

Figure 1

Positive manifestations in examination and in the surgically resected segment. A: A giant and ovoid ulceration in the inferior extremity of the esophagus; B: A typical oval-shaped large ulcer at the ileocecal junction; C: A few oval ulcers around the crissum; D: The resected material showed that the wall of the cecum was thick, occasional discoloration in the serosa, mucosal edema, an ulcer (4 cm × 4 cm) and occasional necrosis in a segment 32 cm in length involving the ileocecal region.

Figure 2

Positive pathological manifestations in the surgically resected segment. A: The ulcer in the ileocecal lesion encroached the whole segment with ectasia and blood vessel hyperplasia [hematoxylin and eosin (HE), × 40]; B: Ectasis in blood vessels was observed in normal tissues around the lesion (HE, × 40); C: The thickened vessel intima with lymphocyte and polymorphonuclear leukocyte infiltration (HE, × 40); D: Extreme ectasia in lesion blood vessels (HE, × 40); E: There was considerable lymphocyte infiltration in and around the vessel wall (HE, × 200); F: Thrombus and recanalization in some vessels at the base of the ulcer (HE, × 100).