Behcet's Disease: Vasculitis

Hulusi Behcet, a Turkish dermatologist first described a disease associating uveitis with genital and oral aphthous lesions, a disease which now bears his name in 1937 (Wechsler and Piette 1199). Fifty years later, Behcet's disease had expanded and was recognized as a chronic, multisystem disorder in which vasculitis was the underlying disorder. The disease occurs in the second to fourth decade of life, and rarely in children and adults over the age of 50 years. During the annual meeting of the Medical Society of Athens in 1930, Benediktos Adamantiades, a Greek ophthalmologist from Prussa in Asia Minor, which is now Bursa, Turkey, gave a lecture on a case of "relapsing iritis with hypopyon" and described a case of a young man with three cardinal signs of the disease (Zouboulis and Kaklomanis 691). He connected the symptoms of genital ulcers, arthritis, and ocular disease as manifestations of a single disease entity, and correlated it with the findings of others. In 1946, Adamantiades added thrombophlebitis as a fourth sign of the disease, and later presented the first classification of the disease, describing the ocular, mucocutaneous and systemic forms. This has led to the adoption of the term Adamantiades-Behcet's disease, since it honors both men who first described the disease and its manifestations.

The etiology of Behcet's disease is not really known (Wechsler and Piette 1199). There may be an infectious cause, with suggestions ranging from streptococci to her

ions or aneurysms, and often requires surgical treatment. The articular manifestations in Behcet's disease are not usually destructive, and for most patients, Behcet's disease is not life-threatening. In children, Behcet's disease can involve intestinal symptoms. Ventura, Trevisiol and Marchetti (2115) reported on nine pediatric cases of Behcet's disease according to the major diagnostic criteria for an International Study Group for Behcet's disease (eye disease, oral aphthous lesions, skin lesions, genital ulcers) which found gastrointestinal symptoms in a low percentage of patients. A Japanese study found gastrointestinal symptoms in 69 percent of children with the disease. Eight patients in the study by Ventura, Trevisiol and Marchetti had recurrent aphthous stomatitis, six had skin lesions, five had genital ulcers, and four had eye disease. Severe gastrointestinal symptoms, including diarrhea, abdominal pain, and perforation requiring surgery, occurred in only one patient. The sensitivity of nuclear medicine procedures for assessing gastrointestinal involvement was assessed in symptom-free patients. The researchers did monoclonal antibody technetium-99 leucocyte scintigraphy in the 8 patients with no gastrointest

Category: Medical - B