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Behcet's, an enigma after decades of research

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Bernice Clark |
Behcet's
Rare disease   From mouth sores to serious brain inflammation

In the last two decades numerous extensive studies have been conducted to reveal the nature of Behcet's disease, a relapsing inflammatory disorder, that is still an enigma for doctors and researchers, which makes it one of the hardest diseases to recognize.

The disease commonly affects men and women in their 20s and 30s, though children and older adults also can develop the condition. It is most common in the Mediterranean and Middle and Far East countries, Turkey having the highest prevalence with 80-420 cases/100.000.

What's the cause or what triggers the disease remins unknown. There may be a genetic cause and some combination of bacteria and viruses, but that's just "may".

To diagnose Behcet's, it heavely depends on the doctor's ability to indentify a group of nonspecific mucocutaneous lesions which also manifest in a number of other diseases. Criteria have been established for the diagnosis of Behcet's disease, but these aren't always essential for the diagnosis of the disease.

Dr. Hulusi Behcet published the paper describing three mayor signs almost eighty years ago, yet its diagnosis criteria, classifications and pathogenesis are still controversial.

What can be said for sure is that nearly everyone with Behcet's will have mouth sores. Then, a patient must have at least two additional signs: genital sores, aye problems, skin sores, and positive pathergy test.

The patients' hospital records covering 16 years follow up data revealed that in some cases Aphthous oral mucosal ulcers (AOUs) had proceeded 10 years a definitive diagnosis being manifesting only as oral aphthas.

Unfortunately, AOUs are neither specific nor rare and they usually heal in one to three weeks. Though they do recur. Mouth sores that recurred three or more times in 12 months are suspicious.

The disease also attacks skin, createing problems with inflammatory response that causes the skin to develop red and tender nodules.

Then, more than 50% of petients of both sexes get genital ulcers. Many sexual transmited diseases manifest with genital ulcers and its observation or remining scars is of diagnostic value. Extragenital ulcers are seen in about 3% of patients.

Patients often experience inflammation of the middle layer of the eyes or the front of the eye. Symptoms include pain, blurry vision, sensitivity to light, and excessive tear production. Those eye problems can cause serious complications if they are left untreated, all the way to blindness.

The disease also causes joint swelling, and an inflammation in blood vessels (veins and arteries). And that can lead to serious complications such as blood clots, aneurysm, and narrowed or blocked vessels.

Behcet's disease may also cause a variety of signs and symptoms that affect the digestive system, including abdominal pain, diarrhea and bleeding.

And finaly, it may cause inflammation in the brain and nervous system. That can cause headaches, confusion, strokes, personality changes, disorientation, fever, poor balance, and memory loss.

The positive pathergy test used to define the cutaneous hyperreactivity to minimal trauma (that is also reporterd in chronic myeloid leukemia (CML) patients), differs between different countries and its studies are conflicting.

Considering available information and the prevalance of Behcet's in certain countries (Middle East and Far East, Turkey, Iran, Japan and China), if we put the nationality into equation, we may be more certain that we are on the right path to the diagnosis.


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