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.     

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.     

Behçet's disease with relapsing cutaneous polyarteritis-nodosa-like lesions, responsive to oral cyclosporine therapy

Cutaneous polyarteritis-nodosa-like lesions are rarely described in Behcet's disease. We report a case of recurrent cutaneous polyarteritis-nodosa-like (C-PAN-like) lesions in Behcet's disease with multiple deep vein thromboses as part of systemic vasculitis. The mucocutaneous manifestations responded to prednisolone; however, C-PAN-like lesions were refractory and responded to oral cyclosporine. We conclude that C-PAN-like lesions may be marker of severe disease and require intense immunosuppressive therapy.     

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.     

Cardiac complications in Behçet's disease

Summary Behçet's disease (BD) is a multisystem disease of unknown aetiology characterized by chronic relapsing oro‐genital ulcers, uveitis, and systemic involvement including articular, gastrointestinal, cardiopulmonary, neurologic and vascular pathology. Vascular involvement is observed in 30% of cases. Although the pathogenic mechanisms underlying the thrombotic disposition in BD are not well known, prothrombin (PT) gene mutations may be one factor that contributes to the development of vascular involvement in this disorder. We report a case of BD with a PT gene mutation, presenting with cardiovascular involvement.     

Possible isotretinoin-induced keloids in a patient with Behçet's disease

Isotretinoin‐induced keloid formation has occasionally been reported in patients who have undergone dermabrasion or laser treatment. This report describes a man with Behçet's disease (BD) who was prescribed isotretinoin for nodulocystic acne. After approximately 8 weeks of this treatment, the patient developed extensive keloids. There are no previous reports of possible isotretinoin‐induced keloid formation in the setting of BD. This article discusses possible connections between BD, keloid formation and isotretinoin, which may be manifested in this case.     

Neutrophilic eccrine hidradenitis in a patient with Behçet's disease

Patients with Beh?et's disease may develop multiple mucocutaneous manifestations, several of which are mediated by neutrophils. These include aphthous ulcers, pseudofolliculitis, acneform lesions, and pathergy. We report another neutrophil-mediated disorder, neutrophilic eccrine hidradenitis (NEH), in a patient with Beh?et's disease. NEH should be added to the list of mucocutaneous lesions that may be seen in patients with Beh?et's disease.     

A case of pediatric Behçet's disease with intestinal involvement

We report a 6-year-old girl with recurrent oral aphthae, genital and perianal ulcers, and folliculitis-like erythema followed by intestinal symptoms. No ocular lesions were found. Her ailment was diagnosed as incomplete Behçet's disease with intestinal involvement. According to the literature, pediatric Behçet's disease is characterizesd by a low incidence of ocular lesions and a high incidence of intestinal involvement, as exhibited in this case. As intestinal lesions in pediatric Behçet's disease are often life-threatening, a barium enema must be given to exclude the possibility of intestinal involvement when pediatric patients with Behçet's disease complain of abdominal pain