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Behcet's Syndrome - Symptoms & Treatment


Behçet's syndrome, (now known as Behçet's disease), is a chronic condition which happens because of disturbances in the body's immune system. It is most common along the "Old Silk Route", which spans the region from Japan and China in the Far East to the Mediterranean Sea, including countries such as Turkey and Iran. It is classically characterized as a triad of symptoms that include recurring crops of mouth ulcers (apthous ulcers), genital ulcers, and inflammation of a specialized area around the pupil of the eye (the uvea). No-one knows why the immune system starts to behave this way in Behçet's disease. It is not because of any known infections, it is not hereditary, it is not to do with ethnic origin, gender, life-style, age, where someone has lived or where they have been on holiday. In Behçet syndrome, the basic lesion is vasculitis. Biopsies have shown vasculitis near lesions of Behçet syndrome, including the oral and genital ulcers and lesions of the CNS and the eyes; large vessels are affected by a vasculitis of the vasa vasorum. The cause of Behcet's syndrome is unknown. Usually, symptoms flare up repeatedly and then disappear. The time between attacks tends to be unpredictable - as short as a few days or as long as years.

Because Behcet's syndrome is a multisystem disease, it is difficult to diagnose. It is virtually unparalleled among the vasculitides in its ability to involve blood vessels of nearly all sizes and types, ranging from small arteries to large ones, and involving veins as well as arteries. The inflammation of the area of the eye that is around the pupil is called uveitis. Ulcers occur elsewhere, as in the gut and on the skin. In addition, an assortment of nonulcerative skin lesions may be present such as folliculitis, erythema nodosum, photosensitivity, an acne-like exanthem and infrequently vasculitis. Behçet's syndrome is rare in the UK. We do not know exactly how many people in the UK have Behçet's syndrome, but there are probably about two thousand. Onset typically occurs in patients in the late third and early fourth decades of life. Onset during the childhood years is well recognized, but Behçet syndrome rarely occurs before school age. Inflammation of the outer fibrous layers of tissue (tunic) that surround the eyes (posterior uveitis) also affects individuals with Behcet's syndrome. Treatment of Behcet's syndrome is symptomatic and empirical.

Causes of Behcet Syndrome

The common causes of Behcet Syndrome include the following:

  • The exect cause of Behcet's syndrome is not known.
  • An infectious agent such as streptococci could play a role in the pathogenesis of the disease.
  • Genetic factors.
  • Viral infections may also a cause of this disease.

Symptoms of Behcet Syndrome

Some sign and symptoms related to Behcet Syndrome are as follows:

  • The mouth and genital ulcers are generally painful and recur in crops (many shallow ulcers at the same time).
  • Pain and irritation in the eyes.
  • Inflammatory bowel disease.
  • Joint pain and swelling, usually in the knees.
  • Fever.
  • Reddish bumps, pimples, or sores on skin.
  • Neurological problems.

Treatment of Behcet Syndrome

Here is list of the methods for treating Behcet Syndrome:

  • Colchicine or azathioprine can be used for systemic manifestations, such as severe ulcers or skin disease.
  • Rest and exercise are also useful in the treatment of Behcet syndrome.
  • Antileprosy drugs, dapsone and thalidomide, and the immune modulator levamisole may be useful.
  • Topical corticosteroids may relieve the pain of oral lesions and lidocaine mouthwash also will alleviate pain.
  • Corticosteroid creams that are applied to the skin may relieve the pain of ulcers. Mouthwashes that contain a local anesthetic, such as xylocaine or lidocaine, may temporarily relieve pain.
  • Thrombotic events are treated with anticoagulants.
  • Gastrointestinal perforations and subsequent peritonitis need surgical treatment.

 

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