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Behcet’s Syndrome

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Behcet’s (beh-CHETS) Syndrome (or Behcet’s Disease) is an autoimmune disorder that causes the inflammation of blood vessels in the body. An autoimmune disorder means that it involves the body’s own immune system attacking its own healthy cells. The symptoms produced by this disease are different for each person, and the inflammations may occur on different parts of the body. The goal of treatments is to reduce the signs and symptoms of Behcet’s Syndrome, and to prevent the occurrence of potential complications like blindness.

Causes

The causes of Behcet’s Syndrome are unknown, but some likely factors include genetics and environmental conditions. It is also believed by doctors that certain viruses or bacterium may trigger the onset of this disorder. There are however, some factors that are known to increases your risk of developing Behcet’s Syndrome:

  • Gender: Tends to be more severe in males
  • Age:  Most common in the age range of 20-40, but it is not impossible for individuals outside of this range to develop this disorder
  • Genes: Specific genes increase the risk of Behcet’s Syndrome
  • Location: This disease is more likely to develop in individuals living in the Middle East and Far Eas

Symptoms

single-oral-aphthous-ulcer

The symptoms associated with Behcet’s Syndrome behave in unpredictable trends. They may or may not become less severe over time. As mentioned earlier, the treatments are aimed at reducing the effects of symptoms, as well as preventing any complications from occurring. The symptoms, which is the inflammation of blood vessels, may occur almost anywhere in the human body. Here are some patterns of Behcet symptoms in some key areas of the body:

Skin:

  • Rashes called “folliculitis” may form almost anywhere, which resemble acne
  • Erythema nodosum, which are skin lesions with a red appearance may develop on the legs and ankles, but in some case can appear on the face, arms, or neck too

Joints:

  • Arthritis may develop, which is joint pain without swelling

Lungs:

  • Inflammation of blood vessel walls of arteries in the lung (Aneurysms), may lead to lung hemorrhage

Mouth:

  • Sores called “aphthous ulcers” can appear in the mouth. These are typically painful if touched, and are a common occurrence for many people, even those without Behcet’s Syndrome. However, the ones caused by Behcet’s are more numerous, frequent, larger, and more sensitive. These sores may develop on the tongue, lip, or inside of the mouth

Genitals:

  • For males, scrotal lesions, that are similar to the ones in the mouth may develop on the scrotum
  • For females, genital ulcers may develop on the vulva

Brain:

  • Behcet’s Syndrome involves the white matter of the brain, which can cause headaches, strokes, dementia, or other personality alterations
  • The protective meninges surrounding the brain may also be affected, which can lead to meningitis

Intestines:

  • Ulcers can develop anywhere in the gastrointestinal tract due to inflammation. This is similar to inflammatory bowel diseases.

 

Diagnosis

It is important to note that a large number of the mentioned symptoms are not unique to Behcet’s Syndrome. This disorder occurs very rarely, so having one of the symptoms may not guarantee that you have this disorder. Before any type of treatment can occur, a doctor or physician must be contacted and they will assess the situation. The widely accepted criteria for diagnosing Behcet’s Syndrome are the occurrence of recurrent oral ulcerations and at least two of the following symptoms:

  • Eye lesions
  • Skin lesions
  • Genital ulcerations
  • Positive pathergy test

The last mentioned test involves pricking the patient with a sterile needle and observing it 48 hours later. If a red nodule forms at this time, the test is deemed positive.

Treatment

Treatments vary from patient to patient because of the large range of symptoms involved with Behcet’s Syndrome. For symptoms that are limited to mucocutaeous regions (mouth, skin, genitals), topical steroids and non-immunosuppressive medications are used. Mouthwash with local anesthetic can be used to relieve pain for oral symptoms, while eye drops can be used for inflammation of the eye.

For serious organ involvement (eye, nervous system), high doses of prednisone and immunosuppressive treatment are typically used instead. If the condition is life threatening, a combination of prednisone and cyclophosphamide are used.


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