PURPOSE: Behcet's disease (BD) is a chronic, relapsing, multi-system vasculitis of unknown aetiology with complicated and diversified clinical features predominantly involving oral and genital ulcers, and ocular and cutaneous lesions. The clinical features of this disease have been described to be different according to geographical areas and gender. We investigated the specific clinical features of BD patients in Northeastern China. MATERIALS AND METHODS: 116 patients involved in this study fulfilled the classification criteria of the International Study Group for BD. The clinical manifestations and results of laboratory tests of BD were recorded in each patient. RESULTS: The onset was typically between 20-39 years with a slight female predominance. Oral ulcers were the most common manifestation, followed by skin lesions, positive pathergy reaction/genital ulcers, and ocular lesions. Vascular lesion and epididymitis were rare in patients with BD. The frequency of erythema nodosum-like lesion and articular involvement were significantly higher in females, while gastrointestinal involvement was significantly higher in males. The results of laboratory tests showed that the human leukocyte antigen (HLA)-B*51 alleles were positive in 30.9% of patients and the immunological abnormities were present in some patients. CONCLUSION: The clinical features of BD showed geographical and gender difference. Genetic and immune factors might participate in aetiopathogenesis of BD.
Behcet Syndrome/*diagnosis/epidemiology/pathology ; China ; Female ; Geography ; Humans ; Male

Behcet disease is relatively rare in pediatric age group. And the bowel involvement is seen in only a small portion of Behcet disease. However, once the bowel is involved it is potentially life threatening event. We report a 15 year old boy with intestinal Behcet's disease who had a history of recurrent oral and genital ulcers for several years. He underwent right hemicolectomy under the impression of intestinal tumor. Pathologically the lesion was a large sharply delineated ulcer in the cecum. The ulcer was round and deep with elevating margin, and was associated with thickening of affected intestinal wall. Microscopically, the ulcer base consisted of granulation tissue with fissurings and underminings. Characteristic phlebitis and occlusive arterial lesion were seen in intestinal wall. The inflammatory lesion was most pronounced around the ulcer but could be recognizable throughout the resected specimen.
Adolescent ; Behcet Syndrome/*diagnosis/pathology ; Diagnosis, Differential ; Humans ; Intestinal Diseases/*diagnosis/pathology ; Male ; Ulcer/pathology

OBJECTIVES: To analyze arthritic manifestations in Behcet disease, which is one of the most common manifestations of Behcet disease. METHODS: Among the patients who visited the Rheumatology Division, Keimyung University Dongsan Medical Center, Taegu, Korea from March 1997 to February 1998, 35 patients, with more than 3 months follow-up, were compatible for the diagnosis of Behcet disease according to the Shimizu criteria, after exclusion of uncertain or possible Behcet cases. The presence of various manifestations was evaluated. Regarding the joint manifestations, the involved joint, signs and the pattern of the articular symptoms were examined. Basic laboratory tests, HLA studies and simple radiologic studies were done. RESULTS: All 35 patients had evident, recurrent, painful oral ulcers by the study definition. Genital ulcers were found in 29%, skin lesions in 77%, uveitis in 9%, gastrointestinal ulcerations in 6% and vascular manifestations in 6%. Joint manifestations appeared in 97%. Knee(91%), proximal interphalangeal (53%) and metacarpophalangeal joints(21%) were the main sites. Tenderness was prominent in 91% and swelling in 44%. Polyarticular presentation was found in 47%. In most cases (76.4%), the articular symptom was short-lasting. C-reactive protein was likely to be positive in active Behcet disease. HLA B51 was positive in 46%. CONCLUSIONS: In Behcet disease, various manifestations can be found. The arthritic manifestation seems quite common. It may present as seronegative rheumatoid arthritis. Otherwise, it may present as palindromic rheumatism.
Adult ; Arthritis, Rheumatoid/diagnosis* ; Arthritis, Rheumatoid/blood ; Behcet's Syndrome/diagnosis* ; Behcet's Syndrome/blood ; C-Reactive Protein/metabolism ; Comparative Study ; Diagnosis, Differential ; Female ; Human ; Joints/pathology ; Male ; Middle Age ; Rheumatic Diseases/diagnosis* ; Rheumatic Diseases/blood

Behcet's disease is a multi-systemic vasculitis and characterized by systemic organ involvement. Although the gastrointestinal and systemic features of Behcet's disease and inflammatory bowel disease overlap to a considerable extent, they are generally viewed as two distinct diseases. A 39-yr-old female was diagnosed as having Behcet's disease. She was admitted to our hospital because of oral and genital ulcer, lower abdominal pain, and frequent diarrhea. Colonosopy showed diffuse involvement of multiple longitudinal ulcers with inflammatory pseudopolyps with a cobblestone appearance and ano-rectal fistula was suspected. These findings are extremely rare in Behcet's disease. However, there were no granulomas, the hallmark of Crohn's colitis. Microscopically, perivasculitis and multiple lymph follicles compatible with Behcet's disease were seen. Although being rarely encountered, multiple longitudinal ulcers, cobblestone appearance, and ano-rectal fistula can develop in Behcet's disease, as in Crohn's colitis. Therefore, Behcet's disease and Crohn's disease may be closely related and part of a spectrum of disease.
Adult ; Behcet Syndrome/diagnosis/*therapy ; Colitis ; Colonoscopy ; Crohn Disease/diagnosis/*therapy ; Female ; Humans ; Inflammation ; Inflammatory Bowel Diseases/pathology ; Intestinal Diseases/diagnosis/*therapy ; Perineum/pathology ; Ulcer ; Vulva/pathology

OBJECTIVETo investigate the clinical features, diagnosis methods, therapeutic principles of intestinal Behcet's disease.

METHODSking Union Medical College Hospital from August 1998 to April 2010 were retrospectively analyzed.

RESULTSThe clinical courses of patients with intestinal Behcet's disease were from 26 days to 33 years, and the average duration was 6.32 +/- 1.01 years. The appearance of extra-gastrointestinal symptoms was significantly earlier than that of gastrointestinal symptoms (7.35 +/- 1.39 years vs. 3.24 +/- 0.82 years, P<0.05). The predominant gastrointestinal manifestations were right lower quadrant pain (95.56%) and hematochezia or melena (40.00%). Misdiagnosis occurred in 17 cases. In patients without systemic medicine therapy before surgery, the incidence of postoperative infection of incision site and abdominal cavity was significantly higher than that in those undergoing systemic medicine therapy (80.00% vs. 0%, P<0.05).

CONCLUSIONSBecause of the diversity of gastrointestinal manifestations, intestinal Behcet's disease is easily misdiagnosed. The systemic medical therapy before surgery could decrease the incidence of infection of incision and abdominal cavity.

Adolescent ; Adult ; Aged ; Behcet Syndrome ; diagnosis ; pathology ; surgery ; Child ; Female ; Gastrointestinal Diseases ; diagnosis ; pathology ; surgery ; Humans ; Intestinal Diseases ; diagnosis ; pathology ; surgery ; Male ; Middle Aged ; Young Adult

A rare case of Behcet's disease associated with myelodysplastic syndrome (MDS) is described. A 50-year-old Korean female suffering recurrent oral ulcer, genital ulcer, fatigue, arthralgia in both knees and fever was diagnosed as Behcet's disease. The findings of bone marrow aspirates were consistent with refractory anemia, a subtype of myelodysplastic syndrome. Chromosomal analysis of bone marrow cells revealed 46,XX,-8,-20,+der(8)t(8;20)(p23;p10),+der(8) t(8;20)(p23;q10)[30]. The chromosomal changes found in this patient were different from those of previous reports, which mostly revealed trisomy 8. If anemia, low reticulocyte count and dyspoietic cells are sustained in Behcet's disease, physicians should be alert to the possibility of MDS with aberration in chromosome 8 and perform a bone marrow study for the proper diagnosis and treatment of the disease. We presented a case of Behcet's disease associated with MDS, which is the first Korean case.
Anemia/genetics ; Behcet's Syndrome/genetics* ; Behcet's Syndrome/diagnosis ; Bone Marrow Cells/ultrastructure ; Bone Marrow Cells/pathology ; Case Report ; Chromosome Aberrations ; Female ; Histocytochemistry ; Human ; Korea ; Middle Age ; Myelodysplastic Syndromes/genetics*

We report an unusual case of unilateral frosted branch angiitis associated with Behcet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behcet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behcet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.
Adult ; Behcet Syndrome/*complications/diagnosis ; Diagnosis, Differential ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Magnetic Resonance Imaging ; Male ; Retinal Artery/*pathology ; Retinal Vasculitis/diagnosis/*etiology

Behcet's disease (BD) has been recognized as multi-systemic chronic vasculitic disorder of recurrent inflammation, characterized by the involvement of multiple organs and resulting in orogenital ulcers, uveitis, and skin lesions. Involvement of the central nervous system, vessels, and intestines in BD often leads to a poor prognosis. Digestive manifestations in BD have been reported in up to 1-60% of cases, although the rate varies in different countries. The most frequent extra-oral sites of gastrointestinal involvement are the ileocecal region and the colon. Gastric or esophageal involvement is reported to be very rare. Moreover, there have been no reports on the simultaneous involvement of the esophagus, stomach, ileum, and colon. Here, we present a 55-year-old Korean man with intestinal BD and multiple ileal and colonic ulcerations complicated by perforation, gastric ulcer with bleeding followed by perforation, and esophageal ulcers with bleeding.
Behcet Syndrome/complications/*diagnosis/pathology ; Cecal Diseases/complications/pathology ; Diagnosis, Differential ; Endoscopy, Digestive System ; Gastrointestinal Diseases/complications/*diagnosis ; Gastrointestinal Hemorrhage ; Humans ; Intestinal Perforation/*diagnosis/etiology/pathology ; Male ; Middle Aged ; Peptic Ulcer Perforation/pathology ; Stomach Ulcer/complications/pathology

The intestinal lesion of Behcet's colitis shows aphthoid or punched-out ulceration. However, the diagnosis of Behcet's colitis should be based on the presence of other stigmata of Behcet's syndrome, since these morphological characteristics are not pathognomonic by themselves. Furthermore, the stigmata of Behcet's syndrome could appear simultaneously or separately with intervals of several months to years. Besides, when a physician first meets patients with intestinal ulcerations of aphthoid or punched-out shape, if they do not have any stigma of Behcet's syndrome, the physician has some difficulty in making a diagnosis of Behcet's colitis. The purpose of this retrospective study was to investigate the followings: 1) The upgrade in clinical type of Behcet's colitis with the advance of time. 2) What portion of the patients with aphthoid or punched-out ulcerations, but without any other clinical feature of Behcet's syndrome, could be diagnosed as Behcet's colitis with the advance of time? During the mean follow-up period of 38.2 months, 4 (22.2%) out of 18 patients with Behcet's colitis upgraded their clinical types. In the nonspecific ileocolitis group, who had no major stigma of Behcet's syndrome on their initial visit, 3 (30%) out of 10 patients were subsequently diagnosed as Behcet's colitis during the mean follow-up period of 33.3 months. From these results, we could conclude that in possible or suspicious cases of Behcet's colitis, a more confident diagnosis could be made by close observations for new developments of major stigma of Behcet's syndrome. Even in cases of nonspecific ileocolitis, the diagnosis of Behcet's colitis could be made in a significant number of cases as time goes by.
Adolescent ; Adult ; Behcet Syndrome/classification/*diagnosis/pathology ; Chi-Square Distribution ; Colitis, Ulcerative/*pathology ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Time Factors

No abstract available.
Adult ; Behcet Syndrome/*complications/diagnosis ; Follow-Up Studies ; Humans ; Male ; Optic Disk/*pathology ; Optic Neuritis/diagnosis/*etiology ; Time Factors ; Visual Acuity