Behcet’s Disease Presenting with an Esophageal Aphthous Ulcer: A Case Report

Behcet’s Disease Presenting with an Esophageal Aphthous Ulcer: A Case Report

Corresponding author: Dr. Maryam Masoumi, Qom University of Medical Sciences, Qom, Iran – Rheumatology Research Center, Tehran, Iran, Email:



Behcet’s disease (BD) is a chronic inflammatory vasculitis which may affect both small and

2large vessels. The disease is mainly characterized by recurrent oral aphthous ulcers, genital aphthous ulcers, skin lesions, uve- itis and articular involvements. Behcet disease’s gastrointestinal involvement consists of mucosal ulcerations of the gastrointes- tinal tract (Gut), abdominal pain, diarrhea, perforation, etc.

Case presentation

A 20-Year-old male was admitted to our clinic for his chest pain. He complained of a present odynophagia also. Diagnostic endoscopy revealed two esophageal lesions with the sizes of 20 mm.However, the past history included other complaints like oral ulcers, genital ulcers, and epididymitis. Further inves- tigations with total GI colonoscopy demonstrated GI vasculitis secondary to BD.


BD may be easy to diagnose when it presents with oral and genital ulcers, or panuveitis. However, presentation with chest pain and odynophagia secondary to esophageal lesions may warrant a thorough investigation. presence of some focal radiopacities.


Behcet’s Disease (BD) is a chronic, multisystemic inflammato- ry disorder. It is rare but the prevalence is high in countries such as China, Korea, Japan, and Turkey and Iran along the an- cient Silk Road from East Asia to Mediterranean, and the lower prevalence of BD is in the United States and Northern Europe- an countries. [1-4]

The disease was first described by the Turkish dermatologist, Dr. Hulusi Behcet. In 1937 he described a triad of oral and gen- ital aphthous ulcerations, and Uveitis. It was classified as a vas- culitis in 2012 in the variable vessel subgroup affecting vessels of all sizes, types, and localizations.

[5] Since then, BD revealed other clinical manifestations in- cluding skin, gastrointestinal, articular, cardiopulmonary, and neurologic involvement. Abdominal pain, GI bleeding, stom- achache and nausea, diarrhea and esophageal stenosis are the GI symptoms in BD patients. They are not common as the other disease’s symptoms and occur due to ulcerations in the gastroin- testinal tract. GI ulcerations are uncommon of various types in the esophagus, stomach, and small and large intestines, as well as deeply penetrating ulcerations in the ileocecal region, with frequently accompanying enteric fistulas. The ulcers around the

ileocecal region are the most common, [6-7] although esophageal aphthous might be a rare and unique lesion [8]. Here we report a young man diagnosed to have BD with chest pain and odynopha- gia due to an esophageal aphthous ulcer.

Case Presentation

A 20-year-old man was admitted to the Emergency Department of our university hospital with a chief complaint of a chest pain that had begun 2 weeks before. He also complained of an accom- panying odynophagia. Nothing specific was found on his Chest X-ray and electrocardiography. On further investigations, he gave us a history of 1-2 painful oral aphthous two to three times monthly, lasting two to three weeks and improving spontaneous- ly. They had a rise in number and frequency since the last year. On physical examination an oral aphthous was detected on the right side of the upper lip.

A one year history of intermittently genital aphthosis and epididymitis was also given and there were two scars of genital aphthosis, accompanying an active one on examination.The pathergy test demonstrated a papule like lesion surrounded by an erythema (positive pathergy reaction). On our ophthalmologic examination, he had mild bilateral posterior uveitis despite no visual disturbance.

Laboratory tests were unremarkable except for increased eryth- rocyte sedimentation rate (ESR) up to 75 mm and a three plus C-reactive protein (CRP).

Therefore, we performed further immunological tests includ- ing antiphospholipids , antineutrophil cytoplasmic antibodies ( C-ANCA and P-ANCA ) ,antinuclear antibodies, venereal

disease research laboratory (VDRL), viral antigens and serology of HBs Ag, HCV Ab and HIV Ab, human leukocyte antibodies ( HLA ) of B-27,B-5 and B-51. We found only HLA B-5 was positive.

Ultrasonography of abdomen and pelvis was normal. Endoscopy performed showed two longitudinal ulcers of size 20 mm, in the middle third of esophagus, with a distance of 33mm from the incisura. Biopsy specimen was taken and sent to the pathology laboratory. (Fig. 1)

Figure 1: Upper gastrointestinal endoscopy showing two longi- tudinal ulcers of size 2cm with irregular borders located 33mm from the incisura.

Colonoscopy findings: Several aphthous like lesions surround- ed by erythema in the distal portion of transverse colon, small discrete oval-shaped ulcers of sizes 6 mm and 5 mm were seen in

the distal portion of Colon and several biopsies were taken. Other parts were normal in mucosal and vascular patterns. Several small round ulcers of sizes about 4 to 5 mm were observed in hepatic flexure. Several discrete oval-shaped ulcers were detected in the Terminal ileum which the largest was of size 6mm Finally, several discrete oval-shaped ulcers of different sizes up to 20 mm from Ileocecal valve were observed. Multiple biopsies were taken.

Figure 2: gastrointestinal colonoscopy showing aphthous like lesions the transverse colon, small discrete 5 and 6 mm ulcers in the distal colon, several 4 to 5 mm small round ulcers in the hepatic flexure, oval shaped ulcers in the terimnal ileum and several discrete oval-shaped ulcers of different sizes up to 20 mm from Ileocecal valve

The pathology revealed edematous appearance in all portions of specimen including terminal ileum and large intestinal muco- sa. The mucosal lymphoid tissue was reported hyperplastic more prominent in ileum with acute capillaritis. Older lesions and superficial lymphoid tissue follicles accompanying erosions were also detected in large intestine. The small vessels showed endothelial swelling, less neutrophilic exudates. Diagnosis was ileocolitis, focal disseminated follicular (Aphthous) type with chronic and acute microangiitis compatible with intestinal lesion of Behcet’s Disease.

According to the International Criteria for Behcet’s disease, our patient was diagnosed to have Behcet’s disease [10]. Three methylprednisolone daily pulses were given. The patient had great sign of improving in symptoms after 4 days. Then, 1 gr cyclophosphamide monthly pulses and 2 mg/kg azathioprine were given with 0.5 mg/kg prednisolone. Cyclophosphamide pulses were given 3 times and Prednisolone doses were gradually tapered afterward. The treatment was successful and the follow-up endoscopies and colonoscopies revealed no Aphthous or any gastrointestinal complications of aphthous after 6 months.


BD is a systemic, chronic, relapsing inflammatory vasculitis with an unknown etiology, affecting arterial and venous vessels of all kinds and sizes.

BD distribution is worldwide, with a higher prevalence in countries alone the “Silk-Route” like Iran, Turkey, and Japan extend- ing from Asia to the Mediterranean basin.

Diagnosis is based on clinical symptoms but the classification criteria like the International Criteria for

Behcet’s Disease (ICBD), may help. Clinical manifestations of BD in the order of prevalence includes oral aphthous (most com- mon), Genital aphthosis, eyes, skin, articular manifestations, vascular lesions and neurological. The disease is characterized by attacks and remissions. The attacks may resemble the preceding or differ, in duration, severity, and different systems in- volved. As like other organs, GI tract may be involved. GI involvement differs among ethnic groups with a wide variation from 4 % of Behcet patients in Greece to 38% of Behcet patients in Australia and only 7/4% of Iranian behcet patients according to an expert review of the disease. [9] Over 40% of these patients have GI complains of abdominal pain, chronic diarrhea, and hematochezia. Acute abdomen can occur too, due to the intestinal perforation of ulcer. The ulcers occur in 1% of BD patients and they are from small and superficial to large and penetrating ulcers. Symptoms of GIBD (GI involvement in BD) vary from retrosternal pain and hematemesis to gastritis, peptic ulcer and acute abdomen depending on their location from esophagus to anus in the digestive tract. [6] GI problems of Behcet’s disease don’t get much attention unless their complications occur. So they are increasing in prevalence.

On the other hand, because of GIBD manifestations similarity to diseases like Crohn’s Disease, Recurrent Aphthous stomatitis and Celiac Disease, BD may be misdiagnosed.

Ideguchi reported that esophageal involvement was found in 9 out of 43 GIBD patients and that the prevalence of esophageal involvement was low, they revealed few severe complications but responded well to medical treatment [6].

However, the initial findings are not frequent and Behcet Disease isn’t in the top of our differential diagnosis for esophageal aphthous. To date, similar cases of esophageal ulcer of BD has been reported mostly from Korea and Japan which the gastroin- testinal BD is higher in prevalence but none from Iran. We have brought some of those cases in the table below.

In conclusion, taking the history of patients meticulously especially in countries on the silk-route, which are supposed to have a higher incidence of BD, will prevent the late diagnosis that lead to complications like bleeding, fistula, perforations in the GI system, and the supportive findings like uveitis, oral and genital ulcerations, enlighten the diagnostic dilemma rapidly and correctly.


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  15. Ji Hyeon Ju, Seung-ki Kwok, Soo-Hong Seo,et al. Successful treatment of life-threatening intestinal ulcer in Behcet’s dis- ease with infliximab: rapid healing of Behcet’s ulcer with in- fliximab. Clinical Rheumathology. 2007, 26: 1383-1385.
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