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Behçet disease was first described by the Turkish dermatologist Halusi Behçet in 1937 as a characteristic triad consisting in the presence of oral ulcers, genital ulcers and ocular vasculitis with hypopion. Behçet associated this condition primarily to a viral aetiology, which is still to be confirmed. It is also known in the scientific literature as Adamantiades–Behçet, because of Benediktos Adamantiades who also made important contributions for the classification of the condition during the 1940s.1 2 Higher prevalence of this disease has been reported in Mediterranean countries and Japan, varying between 5 and 80/100 000 as in Spain and Turkey, according to data from the American Association of Behçet disease, and 2.5/100 000 in Portugal as described by other authors.3 However, its prevalence could be as high as 420/100 000 as described by others in Turkey.4 It is often diagnosed between the third and fourth decades of life but does not follow a defined natural course throughout life. It has been observed by some authors to be much more frequent in men than in women.5
The pathogenesis of the disease is well known.6 However, to confirm the diagnosis, it should be associated with several of the following signs and symptoms: recurrent genital ulcers present in 90% of patients, ocular involvement, dermatological lesions and/or positive “pathergy test” or “Behçetine skin …
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