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: Intestinal Behcet’s disease, Hemorrhage, Skip ulcers

Core tip: We present a patient with fever, abdominal pain and skip ulcers accompanied by lower gastrointestinal hemorrhage. Although the patient had undergone a number of examinations, no diagnosis was made. The patient underwent emergency surgery due to unmanageable lower gastrointestinal hemorrhage. Pathology of the resected bowel containing ulcer lesions indicated ectasia and blood vessel hyperplasia. The patient was diagnosed with incomplete-type intestinal Behcet’s disease (BD). BD can influence any region of the gastrointestinal tract. It is more difficult to diagnose when intestinal BD is accompanied by multiple ulcers in various positions throughout the entire digestive tract.