%0 Journal Article
%T Clinical and Pathological Manifestations with Differential Diagnosis in Beh£żet's Disease
%A Aysin Kokturk
%J Pathology Research International
%D 2012
%I Hindawi Publishing Corporation
%R 10.1155/2012/690390
%X Beh£żet's disease is a multisystemic inflammatory disease of unknown etiology which usually occurs as a trait of symptoms: aphthous stomatitis, genital ulcerations, and ocular disease. At the beginning of the disease the diagnosis is uncertain because of various clinical manifestations and a long period up to the full clinical picture manifestation. Since neither the laboratory data nor the histopathological signs are truly pathognomonic in Beh£żet's disease, the differential diagnosis depends on a careful evaluation of the medical history and meticulous physical examination to detect concomitant systemic manifestations. Sometimes, some laboratory test may help establish the diagnosis. Subspecialty referral to ophthalmology, rheumatology, neurology, and gastroenterology should be considered when indicated. 1. Introduction Beh£żetĦŻs disease (BD) is a multisystemic inflammatory process of unknown etiology, characterized by relapsing episodes of oral aphthous ulcers, genital ulcers, other skin lesions, and ocular lesions. The Turkish dermatologist Hulusi Beh£żet first described the disease in 1937, as the triad of recurrent oral aphthous ulcers, genital ulcers, and uveitis [1]. It can affect nearly every system and organ including ocular, cardiovascular, gastrointestinal, renal, pulmonary, urologic, and central nervous systems and the joints [1¨C4]. It affects people mainly between the ages of 20 to 40. Both genders are usually equally affected [2, 3]. However different male-to-female ratio was recorded in some countries: there is a male predominance in Middle Eastern countries, such as Iraq, Jordan, Saudi Arabia, and Lebanon, while a female predominance is seen in the USA and Britain [4¨C8]. Because there are no specific diagnostic laboratory tests or histopathologic findings, the diagnosis of the disease relies on clinical criteria and often takes several years to establish a definitive diagnosis after the appearance of the initial manifestations. Manifestations of BD are not consistent among patients. Clinical phenotypes are very heterogeneous and evolution of the disease vary due to ethnic, geographical, and individual differences. Moreover, the initial manifestations and the combination of clinical symptoms are very heterogeneous from patient to patient, even within the same ethnic group. Some patients present with only mucocutaneous symptoms, while some suffer from systemic involvement causing serious complications. As there are no pathognomonic clinical findings, various diagnostic criteria and classifications have been proposed during the years.
%U http://www.hindawi.com/journals/pri/2012/690390/