Behçet syndrome

We present a 34-year-old man with a two-year history of aphthous stomatitis, who later developed painful, erythematous nodules on his lower extremities. A pathergy test was positive, and the diagnosis of Beh?et syndrome (BS) was made. It is important for the dermatologist to recognize the wide variety of cutaneous manifestations of this disorder. A pathergy test is a simple diagnostic tool that may assist in making a diagnosis. Case reports of other unusual skin manifestations in BS also are reviewed.     

Behçet's disease

A 29-year-old man presented with oral and genital ulcers, erythematous papules and pustules on his back and chest, and deep vein thrombi. A diagnosis of Beh?et disease was made. Beh?et disease is a relapsing disorder that affects the mucocutaneous surfaces. It presents usually as ulcers on the orogenital mucosae, but can also present as an acneiform eruption. The International Study Group on Beh?et disease has established criteria that consist of oral and genital lesions, ocular involvement, skin findings, and a pathergy. Treatment of choice is colchicine or prednisone.     

Successful thalidomide treatment of severe infantile Behçet disease

Abstract: We describe an 11‐month‐old child with giant ulcers of the buccal mucosa, necrosis of the tongue, abdominal tenderness, and severe diarrhea due to Behçet disease. Treatment with thalidomide resulted in prompt recovery of the mucocutaneous lesions and gastrointestinal manifestations.     

Behçet disease in a child--emphasis on cutaneous manifestations

Abstract:  The diagnosis of Behçet disease is based upon clinical criteria because of the lack of pathognomonic laboratory findings. Recurrent episodes of oral and genital ulcerations, skin lesions, and ocular manifestations are seen. The disease may also involve the central nervous system, gastrointestinal tract and, less frequently, the large vessels. In general, manifestations occur in the third or fourth decade of life and are not common in children. Therefore few data concerning this age group have been found in the literature. In this study we report a child with Behçet disease beginning at 1 year of age whose cutaneous manifestations were exuberant acne-like and folliculitis-like lesions, which were crucial for diagnostic confirmation.     

Behçet disease (incomplete) and cutaneous polyarteritis nosa

A 42-year-old man with a 2-year history of left posterior uveitis and recurrent oral aphthous ulcers presented for evaluation of recurrent, erythematous, subcutaneous, tender nodules on his lower extremities. A biopsy specimen of the nodules showed a medium-sized-vessel vasculitis with a mild and moderate inflammatory cell infiltrate in the septae and lobules of the subcutaneous adipose tissue. These changes were consistent with cutaneous polyarteritis nodosa. Beh?et disease is a multisystem inflammatory disorder that includes recurrent oral aphthous ulcers, and at least two of the following features: recurrent genital ulcers, eye lesions, skin lesions, and a positive pathergy test. Beh?et disease and cutaneous polyarteritis nodosa have rarely been described in conjunction, and the suggestion has been raised that cutaneous polyarteritis-nodosa-like lesions may actually be a cutaneous marker of Beh?et disease.     

Patient Characteristics in Behçet Disease

Background: Behçet disease (BD) is a chronic, inflammatory, multisystemic vasculitic disorder with a wide spectrum of clinical presentations. The highest prevalence is seen in Turkey. Specific diagnostic tools are yet to be discovered; thus, the diagnosis relies on physicians being acquainted with the symptoms and signs of the disease. Objective: To investigate the epidemiologic characteristics of BD and to emphasize the typical clinical and laboratory characteristics. Methods: This was a retrospective analysis of all the BD patients attending the Ankara Numune Education and Research Hospital throughout the years 2001–4. Diagnosis of BD was made according to the International Study Group criteria. A total of 213 patients were evaluated with respect to family history, clinical features, pathergy test, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), antistreptolysin O, and ferritin levels. When assessing disease activity, the active clinical manifestations on the day of the examination were taken into account. Correlations were analyzed between sex and age distribution, age of onset, disease duration, and family history; and between family history and age of onset, pathergy, clinical manifestations, and laboratory parameters. Correlations were also evaluated between pathergy positivity and clinical manifestations, and laboratory parameters. Correlations between activity scores and age of onset, duration, sex, family history, and laboratory data were also analyzed. Results: The female : male ratio was 1.04, and the mean age of onset was 27 years. Family history did not affect age of onset or disease severity. Men presented with more active disease, and there was a weak but positive correlation between disease activity and CRP. No correlation was observed between duration and age of disease onset. The most common clinical presentations were oral aphthous lesions, genital ulcers, and skin lesions. Men more commonly presented with papulopustular lesions, pathergy positivity, and vascular, eye, and renal involvement, and women presented with arthritis/arthralgia more commonly than men. Vascular lesions, ESR, and CRP showed significant relationships with pathergy reaction. Eye involvement was not affected by age of onset. Conclusions: We believe our results indicate that the pathogenesis of BD is multifactorial. Hormonal factors seem to be of some influence, while genetic background and environmental factors seem to be the major contributors. Infections seem to be among the triggering environmental factors. Predisposing genes may affect the influence of environmental factors. Prevalence studies should be carried out periodically in those countries with a high prevalence ofBDto keep up with the changing dynamics of the disease, which may also shed light on the as-yet unknown pathogenesis of BD.     

Complex aphthosis and Behçet's disease

Complex aphthosis is a disorder in which patients develop recurrent oral and genital aphthous ulcers or almost constant, multiple oral aphthae, without manifestations of systemic disease. Beh?et's disease is a multisystem disease characterized clinically by oral and genital aphthae, arthritis, cutaneous lesions, and ocular, gastrointestinal, and neurologic manifestations. This article reviews both disorders, including their clinical and histologic presentations, factors in pathogenesis, and includes an overview of therapeutic modalities.     

Helicobacter pylori and Behçet's disease

BACKGROUND: Recent investigation of the etiology of Beh?et's disease (BD) has focused on heat shock proteins (HSP) which belong to the HSP 60 family. Both the gastric pathogen Helicobacter pylori (HP) and BD may cause ulcers in the gastrointestinal tract and, HP expresses HSP 60. OBJECTIVE: Whether HP is linked to the pathogenesis of BD or not, and to investigate the influence of HP eradication on clinical parameters of BD. METHODS: Patients with BD were divided into two groups. Group I comprised 49 patients and was investigated for HP seroprevalence and compared with age- and sex-matched controls. Group II comprised 20 patients with BD and HP infection diagnosed by serological and endoscopic examinations as well as the rapid urease test (RUT). A 1-week eradication therapy was administered for HP infection. Patients were examined for the course of BD at monthly intervals. Two months after the eradication therapy, patients underwent an endoscopic examination and RUT for eradication control. Seven patients were excluded because of eradication failure. Thirteen patients were evaluated for the influence of HP eradication on clinical manifestations of BD. The number and size of oral and genital ulcers before the eradication and at the end of the follow-up period were compared statistically. RESULTS: HP seroprevalence between patients with BD and controls did not show significant difference. In 13 patients with BD, the number and size of oral and genital ulcers diminished significantly and various clinical manifestations regressed after the eradication of HP. CONCLUSION: HP may be involved in the pathogenesis of BD.     

Acetylator phenotype in Behçet's disease

The purpose of this study was to determine the acetylator status in Behçet's disease (BD) patients and compare it to a matched group of normal individuals. Thirty‐seven healthy volunteers and forty‐one BD patients were included. Detailed history was taken from the patients. HLA‐B51 was determined in the BD patients. In addition, the Clinical Manifestation Index (C.M.I.) was determined for each patient. Pathergy test was also done. After an overnight fast, each control subject and patient received a single oral dose of 100 mg of DDS. A blood sample was collected after 3 hours and the plasma was separated for determination of dapsone/monoacetyldapsone by HPLC. The frequency of slow acetylators in healthy individuals was 70.2%, while the frequency of rapid acetylators was 29.8%. The frequency of slow acetylators in BD patients was 53.7%, while the frequency of non‐acetylators (undetected monoacetyldapsone MADDS in plasma) was 46.3%. There were no rapid acetylators among the BD patients. There was a strong negative association between the acetylator status and the severity of BD. In addition, acetylator status correlated with HLA‐B51, in that BD patients with positive HLA‐B51 were characterized by a very slow or non‐acetylator status. Slow or non‐acetylators had more severe BD. We conclude that BD patients have a unique acetylator status. This finding may have implications for the theories for the pathogenesis of the disease as well as for therapeutic aspects.