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Behcet syndrome

Behçet’s disease, (formerly known as Behçet’s syndrome), is a chronic condition due to disturbances in the body’s immune system. This system, which normally protects the body against infections by producing controlled inflammation, becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects blood vessels, usually the small ones. As a result, symptoms occur wherever there is a patch of inflammation, and can be anywhere where there is a blood supply. (Taken from

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History

Behçet's disease is named after Hulusi Behçet (1889-1948), the Turkish dermatologist and scientist who first recognized the syndrome in one of his patients in 1924 and reported his research on the disease in Journal of Skin and Veneral Diseases in 1936. The name (Morbus Behçet) was formally adopted at the International Congress of Dermatology in Geneva in September 1947.

Benedikt Adamandiades, a Greek ophthalmologist, reportedly described the disease six years before Behçet did. Nevertheless, only Greek scientists refer to the disease Adamantiades-Behçet's or even Adamantiades' disease.

Behçet's disease was probably first described by Hippocrates in the 5th century.

Pronunciation note

Because it contains a cedilla, "Behçet" is frequently wrongly assumed to be French in origin and pronounced with a sibilant "s" sound (as in "satsuma") or soft "ch" (as in "shoe"), with and the "t" incorrectly silenced: "Beshay". Because Hulusi Behçet was Turkish, the correct pronounciation is with a hard "ch", as in "church", and with the terminal "t" sounded: "Bet-chet".

Pathology

The symptoms of Behçet's disease are believed to be caused by an over-active immune system which, without any apparent infections, produces recurrent outbreaks of inflammation in small blood vessels. Common symptoms include mouth ulcers, sore genitals and eye inflammation, and arthritis in older patients, mostly painful but not life-threatening conditions. However, some patients may be unable to work because of the pain and the impaired vision and mobility. In some severe cases, uncontrolled inflammation may lead to blindness, intestinal complications, stroke, and even meningitis, which can be fatal.

This disease usually first strikes patients in their 20s and 30s. It then becomes a fluctuating lifelong disorder with a series of remissions and exacerbations which can be from days to months. Complete remission is rare.

Diagnosis

There is no specific pathological test for Behçet’s disease at present. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks. Other causes for these symptoms have to be ruled out before making the diagnosis. The symptoms do not have to occur together, but can have happened at any time.

There are three levels of certainty for diagnosis:

  1. International Study Group diagnostic guidelines (very strict for research purposes)
  2. Practical clinical diagnosis (generally agreed pattern but not so strict)
  3. 'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)

Read more at Wikipedia.org


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Behcet's Disease (Inflammatory Disease and Therapy) $28.00 IMMUNOLOGY BEHCETS DISEASE $78.99
You Are Not Alone: 15 People with Behcet's $16.99 Behcet's syndrome may respond to infliximab. (Six Cases). : An article from: Skin & Allergy News $5.95
Gale Encyclopedia of Medicine : Behcet's syndrome $2.30 Complex aphthosis easily mistaken for Behcet's.(Skin Disorders) : An article from: Family Practice News $5.95
Look past ulcers to diagnose Behcet's, aphthosis.(Clinical Rounds) : An article from: Skin & Allergy News $5.95 Behçet's syndrome: Clinical and immunological features : proceedings of a conference sponsored by the Royal Society of Medicine, February, 1979 $72.00
Behcet's Disease $250.00 Behcet's Disease : A Guide to its Clinical Understanding. Textbook and Atlas $181.97

Some Historical Notes on Behcet's Disease
To the Editor: I read with interest the review article (August 2000) on intracardiac thrombus in Behcet's disease (BD) by Mogulkoc et al.[1] Having ...
Intracardiac Thrombus in Behcet's Disease - )
A Systematic Review Background: Intracardiac thrombus formation is a rare but serious complication of Behcet's disease. We aimed to review the clinical ...
Questions and Answers About Behcet's Disease - Pamphlet
This fact sheet contains general information about Behcet's (buh.SETZ) disease. It describes what Behcet's disease is and how it may develop. It also ...
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Abstract The 657del5 mutation of the NBS1 gene has been demonstrated in most patients with Nijmegen breakage syndrome (NBS). We identified four Turkish ...
Behcet disease, Adamantiades-Behcet disease, or Hippocrates-Adamantiades-Behcet disease? - Letter to the Editor
To the Editor: Tirilomis (December 2001) (1) was correct in stating that the first description in contemporary times of Behcet disease was made by ...
Some more historical notes on Adamantiades-Behcet's disease - Letter to the Editor
To the Editor: I read with interest the review article (August 2000) on intracardiac thrombus in Behcet's disease by Mogulkoc et al (1) and the historical ...
Cardiac Thrombus in Behcet Disease - Letter to the Editor
To the Editor: We read with interest the review by Mogulkoc and colleagues (August 2000)[1] regarding intracardiac thrombus in 24 patients with Behcet ...
Vaginal pain and fever in a premenarchal girl: how would you treat?
A 13-year-old girl is brought to your office by her father because she has had vaginal discomfort for 2 days. She also has had a fever up to 104 [degrees]F ...

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