behcet 's disease is a systemic vasculitis characterized by recurrent oral and genital ulcers and ocular inflammation , and may involve the joint , skin , central nervous system and gastrointestinal tract .
there is no specific test for behcet 's disease , and the diagnosis is based upon clinical criteria . although multiple diagnostic criteria have been established , there is no universally accepted definition of behcet 's disease .
the prevalence of intestinal behcet 's disease has been variable in several reports and accounts for about 1 - 2% of behcet 's disease ( 1 , 2 ) .
the clinical symptoms are wide and include anorexia , vomiting , dyspepsia , diarrhea , and abdominal pain .
the typical endoscopic findings are segmental mucosal inflammation , punch - out , fissuring or aphthoid ulcers in the ileocecal area ( 3 , 4 ) .
although strictures are unusual , transmural inflammation and fistulae are frequently observed ( 5 ) .
these findings are similar to those of crohn 's disease . however , longitudinal ulcers , cobblestone appearance , and granuloma formation are very rare findings in intestinal behcet 's disease .
we experienced a rare case of systemic behcet 's disease with intestinal involvement similar to crohn 's colitis .
a 39-yr - old female presented with recurrent oro - genital ulcers , erythema nodosum , and arthralgia .
two years later , she was admitted to our hospital because of oral and genital ulcer , lower abdominal pain , and frequent diarrhea for 15 days .
she had suffered from intermittent hematochezia and cramping abdominal pain for the previous two years .
further history taking revealed she had a post - traumatic epilepsy by traffic accident 13 yr before and had taken anti - epilepsy drugs , carbamazepine and sodium valproate . on admission ,
her blood pressure was 90/60 mmhg , her pulse rate was 70/min , body temperature was 36.5 , and respiration rate was 20/min . her abdominal pain was located in the bilateral lower quadrants without rebound tenderness .
she appeared chronically ill and reported that her stools were maroon , followed by mucoid . on examination of external genitalia , a linear to ovoid shaped
laboratory values showed a white blood cell count of 8,600/l , hemoglobin of 9.1 g / dl , and a platelet count of 172,000/l .
her liver function test showed aspartate transaminase of 13 iu / l , alanine transaminase of 6
iu / l , albumin of 2.3 g / dl , alkaline phosphatase of 357 u / l , and total bilirubin of 0.2 mg / dl .
the other laboratory findings showed fast blood sugar of 119 mg / dl , blood urea nitrogen of 5.2 mg / dl , creatinine of 0.8 mg / dl , sodium of 136 meq / l , and potassium of 3.3 meq / l .
an esophagogastroduodenoscopy ( egd ) was performed to rule out the presence of a massively bleeding upper gastrointestinal lesion .
colonoscopic examination revealed grossly normal - appearing mucosa in the rectum and the sigmoid colon .
however , descending , transverse , and ascending colon , including the cecum , showed multiple longitudinal ulcers and inflammatory pseudopolyps with a cobblestone appearance .
the ileocecal valve and terminal ileum were preserved from the inflammation . a suspicious fistular opening just above the anus
microscopically , specimens from ulcers of the colon showed shallow ulcerations with inflammatory infiltration consisting of lymphocytes and plasma cells .
under sterile condition , intradermal injection of the skin with a 20-guage needle was done . within 48 hr ,
histological examination of the perineal lesion revealed chronic ulcer with acute and chronic inflammatory cell infiltration and increased small blood vessels ( fig .
total parenteral nutrition with nothing per oral and intravenous administration of methylprednisolone , 62.5 mg were started .
treatment with prednisone , 40 mg and 5-aminosalicylate ( mesalazine ) , 4.0 g by mouth was started .
the abdominal symptoms and vulva ulcer improved gradually , and she was discharged 2 months after admission .
behcet 's disease was originally described in 1937 and characterized by oral and genital ulceration and ocular inflammation ( 6 ) .
the diagnosis is clinical and is now based on criteria suggested by an international study group for behcet 's disease ( 7 ) . in the present case ,
oro - genital ulceration and skin lesions led to the diagnosis of behcet 's disease .
the etiology remains unknown , but the most widely held hypothesis of pathogenesis is that a profound inflammatory response is triggered by an infectious agent in a genetically susceptible host ( 6 ) .
other possible mechanism is that behcet 's disease may be autoimmune in origin ( 8 , 9 ) .
an inflammatory response to several autoantigens is found , and generalized aberrant t cell responses results in enhanced nonspecific inflammation .
an increased production of interferon gamma ( ifn- ) by t cells has been demonstrated in active behcet 's disease , and circulating t cells have the t - helper phenotype ( th1 ) predominantly ( 10 ) . in crohn 's disease , murine and human studies have demonstrated an increased expression of th1 cytokines by lamina propria lymphocytes ( 11 ) .
oshima et al . ( 12 ) reported that over 40% of behcet 's disease patients had gastrointestinal complaints .
this so - called intestinal behcet 's disease accounts for only 1 - 2% of cases ( 1 , 2 ) . in intestinal behcet 's disease , ulceration of the gastrointestinal tract
can be found throughout the intestine , but the most frequent area of involvement is the ileocecal region .
behcet 's colitis can appear as ulcerative colitis or crohn 's disease when there are skip lesions with rectal sparing .
the ulcers are usually large , discrete , punch - out appearing lesions and can extend to the serosa .
formation of fistula , hemorrhage , or perforation occurs in up to 50% of cases involving the intestine .
the lymphocytes are infiltrated , and dense perivascular infiltrate is frequently seen ( 3 , 4 ) .
there are many extra - intestinal findings of crohn 's disease , such as oral and genital ulcers , erythema nodosum , uveitis and arthritis , resembling the manifestations of behcet 's disease .
it is also very difficult to distinguish the intestinal behcet 's disease from that of crohn 's disease in some patients .
it is possible that a patient with crohn 's disease meets the criteria for behcet 's disease .
there are several reports on the coexistence of behcet 's disease and crohn 's disease ( 14 , 15 ) . in the present case , we made the diagnosis of behcet 's disease as described above .
however , the gastrointestinal manifestation of this case is quite atypical for behcet 's colitis and more similar to crohn 's colitis - longitudinal ulcers , cobblestone appearance , and ano - rectal fistula are usual findings in crohn 's colitis .
however , we can not conclude that the patient had crohn 's disease because granulomas , the hallmark of crohn 's colitis , were absent .
the findings in the family reported by yim and white ( 16 ) , suggest that inflammatory bowel disease and behcet 's disease may be closely related and part of a spectrum of disease rather than distinct disease entities . in our patient ,
patient 's bowel symptoms , endoscopic appearance , and the response to medical treatment were compatible with crohn 's colitis .
these findings suggest that behcet 's disease may be a part of the spectrum of chronic inflammatory bowel disease .
 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 . 
INTRODUCTION
CASE REPORT
DISCUSSION