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Erectile Dysfunction in Behcet's Disease

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      Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions.

       Erkan Alpsoy (2016)
      Behçet's disease (BD) is a chronic, relapsing, inflammatory multisystem disease of unknown etiology. Oral ulcers, genital ulcers, cutaneous lesions, and ocular and articular involvement are the most frequent features of the disease. Mucocutaneous lesions are considered hallmarks of the disease, and often precede other manifestations. Therefore, their recognition may permit earlier diagnosis and treatment with beneficial results for prognosis. BD is particularly prevalent in "Silk Route" populations but has a global distribution. The disease usually starts around the third or fourth decade of life. Sex distribution is roughly equal. The diagnosis is based on clinical criteria, as there is no pathognomonic test. Genetic factors have been investigated extensively, and association with human leukocyte antigen (HLA)-B51 is still known as the strongest genetic susceptibility factor. The T-helper 17 and interleukin (IL)-17 pathways are active, and play an important role, particularly in acute attacks of BD. Neutrophil activity is increased in BD, and the affected organs show a significant neutrophil and lymphocyte infiltration. HLA-B51 association and increased IL-17 response are thought to play a role in neutrophil activation. Treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents. Although treatment has become much more effective in recent years with the introduction of newer drugs, BD is still associated with considerable morbidity and increased mortality. Male sex, younger age of onset and increased number of organs involved at the diagnosis are associated with a more severe disease and, therefore, require more aggressive treatment.
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        Behçet's syndrome.

        Behçet's syndrome is a systemic vasculitis with an unknown etiology affecting the small and large vessels of the venous and arterial systems. At least two clusters of disease expression have been described. The first includes superficial vein thrombosis, deep vein thrombosis, and dural sinus thrombi. The second includes acne, arthritis, and enthesitis. The presence of these clusters suggests there may be more than one disease mechanism operative in this complex disorder. Recent European League Against Rheumatism guidelines are useful for the management of the disease in organ systems distinct from the vascular, neurological, and gastrointestinal systems. This is because of a lack of controlled studies evaluating such vascular, neurological, and gastrointestinal complications.
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          Behçet's disease in India: a clinical, immunological, immunogenetic and outcome study.

          The clinical and laboratory profile of 58 consecutive patients satisfying the ISG 1990 criteria for the diagnosis of Behçet's disease was analysed. It appears that Behçet's disease in India is predominantly 'mucocutaneous' and 'arthritic'; 'ocular' and 'neuro' Behçet's being uncommon. In comparison to published literature, the onset of disease in this part of the world is significantly delayed. The pathergy test is rarely positive. There is no significant difference in clinical presentation and laboratory investigations between children and adults with this disease; also, no sex difference was observed. A combination of oral steroids and colchicine gives good relief in most cases. Preliminary observations seem to reflect no definite association of any known class I antigen to disease in this part of the world. A detailed study on immunogenetics is underway.

            Author and article information

            From the Department of Dermatology, Venereology and Leprology, Maharishi Markandeshwar Medical College and Hospital, Solan, Himachal Pradesh, India
            [1 ] Department of Medicine, Maharishi Markandeshwar Medical College and Hospital, Solan, Himachal Pradesh, India. E-mail: shuhulmasti@
            Indian J Dermatol
            Indian J Dermatol
            Indian Journal of Dermatology
            Medknow Publications & Media Pvt Ltd (India )
            Mar-Apr 2017
            : 62
            : 2
            : 217-219
            Copyright: © 2017 Indian Journal of Dermatology

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