Original ArticlesBehçet’s disease: an enigmatic vasculitis
Section snippets
Epidemiological features
The disease affects the population of Far Eastern countries, eastern Mediterranean countries, and Japan, the population deriving from the ancient Silk Road. Familial occurrences have been reported from endemic areas.4 The disease occurs more often in men than in women. The male–female ratio is 3:2.5
The prevalence of the disease differs around the world; according to statistical data, 0.3 per 100,000 population in the United States; 0.6 per 100,000 in Great Britain; 7–8.5/100,000 in Japan;
Criteria for diagnosis
Numerous committees have created criteria—for example, Behçet’s Syndrome Research Committee of Japan; O’Duffy Criteria in the United States; Turkish Criteria of Dilşen; Mason Barnes in England; Zhang Criteria in China. In general, the International Study Group (ISG) criteria are the basis for diagnosis of Behçet disease (Table 1).11
These criteria omit the less certain and less common features of the disease. Behçet’s disease is now recognized as a multisystem disorder by articular, vascular,
Etiology
The etiology of Behçet’s disease is uncertain. Genetic, environmental, virologic, bacterial, and immunologic factors have been proposed as causative agents.
Oral aphthous ulcerations
Oral aphtha is considered as the most important manifestation of Behçet’s disease: According to the classification criteria aphthous ulcerations are keystones to the diagnosis. The ulcers appear on the buccal mucosa, tongue, gingiva, and the soft palate. They are classified as minor or major aphthous ulceration and herpetiform ulceration. Minor ulcers are superficial with a diameter of 2 to 6 mm and appear as multiple lesions, developing within 1 to 2 days. They heal without scarring within 7
Clinical course
Behçet’s disease has a highly variable clinical course with recurrences and remissions. In the absence of neurological, vascular involvement, the disease is generally benign and with a rather good prognosis. Blindness, which occurs in up to 25% of the patients, is the major cause of permanent disability.45, 46, 49
Laboratory findings
Patients with active disease may generate an acute-phase response leading to a raised erythrocyte sedimentation rate (ESR), increased serum levels of C-reactive protein (CRP), and elevated plasma complement components such as C3, C4, C9, and Factor B.
A positive pathergy test is the development of cutaneous nodular pustules at a site of trauma after approximately 24 hours. This test can vary between geographical areas.
The common histopathological lesion in all organ systems is vasculitis. The
Treatment
Drugs that have been used include colchicine, nonsteroidal anti-inflammatory drugs, corticosteroids, dapsone, thalidomide, penicillin, acyclovir, and interferon-α.
Colchicine is an alcoloid. It has been accepted as an effective agent in Behçet’s disease, especially for the mucocutaneous lesions and also ocular and articular manifestations of the disease. According to our experience, it gives the best results, increasing the quality of life with the least risk and side effects.51
Corticosteroids
References (52)
- et al.
Behçet disease (Behçet syndrome)
Semin Arthritis Rheum
(1979) - et al.
Antibodies to endothelial cells in patients with Behçet’s disease
Clin Immunopathol
(1993) - et al.
Behçet’s disease
J Am Acad Dermatol
(1990) - et al.
Cutaneous vasculitis in Behçet’s diseaseA clinical and histopathological study of 20 patients
J Am Acad Dermatol
(1997) - et al.
Arachidonic acid metabolites and colchicine in Behçet’s disease
Prostaglandins Leukot Essent Fatty Acids
(1991) Ueber rezidiverende aphtoese, durch ein virus verur sachte Geschwuere am mund am Auge und an den Genitalien
Dermatol Wochenschr
(1937)Description of Behçet’s syndrome in the Hippocratic third book of endemic diseases
Br J Ophthalmol
(1956)- et al.
Familial Behçet’s disease
Jpn J Ophthalmol
(1978) - et al.
Behçet’s diseaseReport of 41 cases and review of the literature
Medicine (Baltimore)
(1975) Summary of International Symposium on Behçet’s disease
J Rheumatol
(1978)