BEHCET’S SYNDROME

Behcet’s syndrome is a disease with no identifiable cause that involves the blood vessels and can involve the entire body. It is labeled as a syndrome because there is no single symptom that can diagnose such; instead, it is made up of a constellation of symptoms. The three manifestations presenting simultaneously include mouth ulcers or sores (the most common), but this does not produce scarring of the skin overlying the oral cavity, irritation, or redness of the eyes and lesions on the genital area among others.

TYPES

Behcet’s Syndrome involves almost all parts of the body. It includes the most extensive organ system, which is the skin down to the smallest blood vessel of the body. 

SYMPTOMS

Symptoms of Behcet’s Syndrome are as follows and are tabulated opposite the organ system involved.

  • Oral cavity– Presents as a painful sore located on the inside of the cheeks.
  • Eyes– Sudden vision loss following eye irritation, redness, and blurring of vision
  • Respiratory – Can present with dyspnea or difficulty of breathing, sharp stabbing chest pain usually associated with a deep breath, and coughing out of blood (this occurs in severe cases where there is already involvement of the pulmonary vasculature).
  • Cardiovascular– Associated with acute (brief duration) dull ache located at the back of the sternum (bone situated in the middle of the chest).
  • Gastrointestinal– Non-specific abdominal pain, a sensation of fullness, loose stools that may be accompanied by blood
  • Joints– Pain is usually felt on the area where two bones meet, preferentially on those areas which carry the most significant weight (knee and thigh bone) 
  • Central Nervous System (brain and spinal cord)- Can present with hearing disturbances, difficulty in concentration and the most severe is seizure ( repetitive, uncontrolled movements of both upper and lower extremities)
  • Blood vessels– Blockage of the arteries and veins, the formation of abnormal tributary or branches

DIAGNOSIS

Unlike the other autoimmune diseases that have serologic testing to detect its presence or absence, Behcet’s Syndrome relies on the presentation of the patient in question. 

Three (3) episodes of oral sores or ulcers within one year with at least 2 of the following: 

  • Eye irritation with the presence of blurring of vision or sudden vision loss
  • Ulcers on the private parts or reproductive areas (lesions on this area causes scarring), Pain on the testicular area in males
  • Skin testing- defined as an induration of >2mm, a day or two after pricking the skin with a g 26 needle. This test has high specificity but, in some ethnicity, yields a false negative result.
  • Presence of lumps and bumps in areas where the hair grows (folliculitis), far too many pimples among people who have surpassed their adolescent years.

TREATMENT

Treatment for Behcet’s Syndrome is geared towards preventing the body from attacking its own. The following treatment options are available though some of them are still on the process of being studied on their indication.

  • Steroids– These types of drugs are known to affect slowing down the hyperactivity of the immune system.
  • Anti- Tumor Necrosis Factor (Anti TNF) can lessen the symptoms associated with ocular symptoms of Behcet’s Syndrome.
  • Thalidomide, an anti-neoplastic drug when administered to patients with such syndrome, reduces the activity of natural killer cells of the body and prevents them from attacking the body.
  • Intravenous Immunoglobulin– An expensive alternative to treatment but is reserved for severe cases.

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