Familial Behçet's syndrome

Behçet's syndrome is reported in a family of which four generations have so far been affected. The index patient also exhibited an unusual schizo-affective disorder. The histocompatibility antigen haplotype 1–17 was common to four with the disease. Genetic transmission may be responsible for the familial nature of the syndrome and the associated schizophrenia.     

Sweet's syndrome associated with Behçet's disease

A case of acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with Behçet's disease occurring in a 48 year old woman is reported. She fulfilled the diagnostic criteria for both Sweet's syndrome and Behçet's disease. There have been several reports of this association. Herpes simplex virus was isolated from a genital ulcer in our patient. The possible role of herpes simplex virus in this interesting association of Behçet's disease and Sweet's syndrome is discussed and a review of the literature is made.     

Histopathologic features of the spontaneous pustular lesions of Behçet's syndrome

Background Papulopustular lesions are common in Behçet's syndrome (BS). A debate exists as to whether they are different from ordinary acne. This has important clinical and pathophysiologic implications.
Objective The aim of this study was to evaluate the histopathologic features of papulopustular lesions seen both in BS and acne vulgaris (AV).
Methods Representative samples were obtained from 17 patients with BS and six patients with AV. The detailed histopathologic features were evaluated by a dermatopathologist blinded to the diagnoses.
Results Considering the epidermis, follicle epithelium, and dermis separately, no differences could be found between the two groups.
Conclusions The papulopustular lesions seen in these two disorders cannot be distinguished on the basis of clinical and histopathologic findings.     

Mucosal presentation of Behçet's disease

Behçet's disease is a multisystem disease with both mucosal and systemic presentations, infrequently seen in the UK and Northern Europe. We present the case of a 23-year-old Caucasian girl who presented with mucosal ulceration and in whom diagnosis was assisted by HLA testing.     

Use of Colchicine in the Treatment of Behçet's Disease

ABSTRACT: To date, various treatment modalities have failed to produce consistent improvement in patients having Behçet's disease. Recent reports in the literature, however, have shown promising results with the use of colchicine. Our patient, an 18-year-old white woman, satisfied the 1977 Istanbul Conference criteria for Behçet's disease by exhibiting ocular inflammation and a five-year history of severe, recurrent oral and vaginal ulcerations. She was treated with 0.6 mg of colchicine orally twice daily, and at the end of two weeks of treatment, the oral lesions had completely resolved, and the vaginal ulcers were reduced in diameter and depth to at least one half. Within one month, all oral and Vaginal ulcers were completely healed.     

Upper gastrointestinal abnormalities and Helicobacter pylori in Behçet's disease

Background  There are few published clinical trials concerning upper gastrointestinal (GI) involvement in Behçet's disease (BD), and most have been performed on patients with upper GI tract symptoms.
Aim  We sought to determine whether routine endoscopy is indicated in asymptomatic patients with BD and whether Helicobacter pylori plays a role in the pathogenesis of BD.
Methods  Forty consecutive patients with BD and 40 age- and gender-matched controls with tinea pedis were studied. All patients underwent fiberoptic esophagogastroduodenoscopy. Urea breath test was used to identify H. pylori .
Results  Abnormalities were noted in 37 patients (93%): hiatal hernia (53%), antral gastritis (33%), pan-gastritis (23%), gastric ulceration (8%), and duodenal ulceration (8%). Helicobacter pylori was found in 26 patients (65%) with BD and in 28 controls (70%) (no significant difference by chi-squared test, P  > 0.05). We found a high incidence of upper GI abnormalities in BD, but the abnormalities were not specific for the disorder.
Conclusions  Routine endoscopy and screening for H. pylori infection may not be necessary in asymptomatic patients with BD.     

Clinical Analysis of 40 Cases of childhood-Onset Behçet's Disease

Abstract: Behçet's disease is a chronic, relapsing, idiopathic, multisystem condition characterized by mucocutaneous, ocular, gastrointestinal, articular, vascular, urogenital, and neurologic involvement. Usually the onset occurs between 20 and 30 years of age; it is very uncommon in children. We analyzed 40cases of childhood-onset Behçet's disease. The male: female ratio was 0·67 the most frequent major sign was oral ulceration, appearing inall patients. Other major sings were genital ulcers (33 patients) skin lesions (29), and ocular lesions (11). the average time interval between the initial oral ulceration and the second major manifestation, the third and fourth features rapidly developed within one to two years. The most frequent minor sign was arthritis, occurring in 11of 40 patients. Intestinal, neurologic, and renal involvement was also present. From our results, we concluded that oral ulceration, which is the most common initia manifestation, should not be neglected in children, since it may signal Behçet's disease.     

Sister chromatid exchange and micronucleus studies in patients with Behçet's disease

Background:  Genetic factors that predispose individuals to Behçet's disease (BD) are considered to play important roles in the development of the disease. The aim of this study was to determine, by counting sister chromatid exchange (SCE) and micronucleus (MN) frequencies, whether DNA damage have an effect on the pathogenesis of BD. Furthermore, our aim was to show if there is an association between oxidative stress and chromosome instability in BD.
Methods:  We analyzed lymphocytes from patients with BD (16 in active and 14 in inactive periods) and 20 healthy controls for SCE and MN frequencies. In addition, malondialdehyde (MDA) level, superoxide dismutase (SOD) level, glutathione peroxidase (GSH-Px) activity, erythrocyte sedimentation rate (ESR) and polymorphonuclear leukocyte (PMNL) count were determined in the all subjects.
Results:  The SCE and MN frequencies were significantly higher in both the active and inactive period patients than in the controls (p < 0.00001, p < 0.0001, p < 0.01 and p < 0.05, respectively), and the MDA level was significantly higher in both the active and inactive period patients than in the controls (p < 0.01 and p < 0.05, respectively). In contrast, the SOD and GSH-Px levels were significantly lower in both the active and inactive period patients than in the controls (p < 0.01, p < 0.05, p < 0.01 and p < 0.05, respectively).
Conclusions:  Our results suggest that increased plasma MDA level and decreased plasma GSH-Px and SOD levels reflect the increased levels of oxidative stress in BD patients, and this situation may impair genetic stability in BD patients.     

Histological and direct immunofluorescence study of cutaneous hyperreactivity in Behçet's disease

The mechanism of skin hyperreactivity (pathergy) in Behcet's disease is unknown. It has been suggested that the response is due to an Arthus-like reaction. In 18 patients with active Behçet's disease, pathergy was induced and the skin biopsied after 24 h. The histological reaction was that of mild to intense round cell infiltration, perivascular in type. Direct immunofluorescence failed in all 18 patients to demonstrate the local presence of immunoglobulins and complement at the site of lesion. A marked increase in the number of mast cells was observed at the site of reaction and scattered throughout the dermis, the role of which is to be further investigated. The study failed to demonstrate a humoral mechanism in the production of the cutaneous hyperreactivity.     

Colchicine in the treatment of the cutaneous manifestations of Behçet's disease

Five patients with Behçet's disease were satisfactorily treated with colchicine. Oral aphthosis, erythema nodosum-like lesions and genital erosions improved greatly within a month as did laboratory findings. We believe colchicine to be the first choice in the management of the cutaneous and ocular lesions of Behçet's disease.     

Serum β2 microglobulin in recurrent aphthous stomatitis and Behçet's syndrome

β₂Microglobulin (β₂m), a constituent of cell surface histocompatibility antigens, was measured in scrum from twenty-eight patients with recurrent aphthous stomatitis, twenty-four patients with Behçet's syndrome and twenty-eight matched controls. Serum β₂m concentrations were significantly greater in recurrent aphthous stomatitis and in Behçet's syndrome than controls, but failed to differentiate the two diseases.