Behçet's disease (BD, or Behçet's syndrome) is a rare, multisystem disease with chronic inflammation of blood vessels (i.e. vasculitis). It is also known as Silk Road Disease. Inflammation is the first response of the immune system to infection or irritation and may be referred to as the innate cascade. ... The arterial system The blood vessels are part of the circulatory system and function to transport blood throughout the body. ... In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammation of the wall of blood vessels. ...

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. An eponym is a person, whether real or fictitious, whose name has (or is thought to have) given rise to the name of a particular place, tribe, discovery, or other item. ... Hulusi Behçet Hulusi Behçet, born on February 20, 1889 in Istanbul, was a Turkish dermatologist and scientist. ... Dermatology is a branch of medicine dealing with the skin, its structure, functions, and diseases (from Greek derma, skin), as well as its appendages (nails, hair, sweat glands). ... This article is about the profession. ... Geneva: the Mont Blanc bridge over the Rhône River and St Peters Cathedral Geneva (French: Genève) is the second-most populous city in Switzerland located where Lake Geneva (French: Lac Léman, but the Genevois are fond of calling it Lac de Genève) empties into the...

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. An optical refractor in use. ...

Pathology

Behçet's disease is believed to be caused by an over-active immune system which, without any apparent infections, produces recurrent outbreaks of inflammation in small blood vessels. As a result, symptoms can occur anywhere in the body with a blood supply. 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 become fatal. The immune system is the organ system that protects an organism from outside biological influences. ... An infection is the detrimental colonization of a host organism by a foreign species. ... A mouth ulcer or canker sore is a painful open sore inside the mouth caused by a break in the mucous membrane. ... A sex organ, or primary sexual characteristic, narrowly defined, is any of those parts of the body (which are not always bodily organs according to the strict definition) which are involved in sexual reproduction and constitute the reproductive system in an complex organism; namely: Male: penis (notably the glans penis... An eye is an organ that detects light. ... Arthritis (from Greek arthro-, joint + -itis, inflammation) is a group of conditions that affect the health of the bone joints in the body. ... Blindness can be defined physiologically as the condition of lacking sight. ... In medicine (gastroenterology), irritable bowel syndrome (IBS) is a group of functional bowel disorders which are fairly common and make up 20–50% of visits to a specialist. ... A stroke or cerebrovascular accident (CVA) occurs when the blood supply to a part of the brain is suddenly interrupted by occlusion (an ischemic stroke- approximately 90%of strokes) or by hemorrhage (a hemorrhagic stroke - approximately 10% of strokes). ... Inferior view of a brain with meningitis caused by Haemophilus influenzae. ...

This disease usually first strikes patients in their 20s and 30s. Then, it becomes a lifelong disorder that proceeds in a series of remissions (periods of apparent good health, usually a few months) and exacerbations (periods of active disease and progression, can be a week to a month). Complete remission is rare. Remission is the state of absence of disease activity in patients with known chronic illness. ...

Treatment

There is no cure yet. Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system.

Epidemiology

Behçet's disease is considered more prevalent in the areas surrounding the old silk trading routes in the Middle East and in Central Asia. Thus, it is sometimes known as Silk Road Disease. However, this disease is not restricted to people from these regions. The Silk Road (Traditional Chinese: 絲綢之路; Simplified Chinese: 丝绸之路; pinyin: sī chóu zhī lù, Persian language راه ابریشم Râh-e Abrisham) was an interconnected series of routes through Southern Asia traversed by caravan and ship, and connecting [Changan, China with Antioch, Syria, as well as other points. ... A map showing countries commonly considered to be part of the Middle East The Middle East is a region comprising the lands around the southern and eastern parts of the Mediterranean Sea, a territory that extends from the eastern Mediterranean Sea to the Persian Gulf. ... Map of Central Asia outlined in orange showing one set of possible borders Central Asia located as a region of the world Central Asia is a vast landlocked region of Asia. ...

An estimated 15,000 to 20,000 Americans have been diagnosed with this disease. In the UK, it is estimated to have about 2 cases for every 100,000 people.

Males are affected more frequently than females.

External links

• Signs & Symptoms of Behçet's disease (http://www.hulusibehcet.net/manifest.htm) (with pictures)
• American Behçet's Disease Association (http://www.behcets.com/home.ivnu)
• Behçet's Syndrome Society (http://www.behcets-society.fsnet.co.uk/index.html) (UK)
• International Society for Behçet's Disease (http://www.behcet.ws/)
• Behçet's syndrome (http://www.whonamedit.com/synd.cfm/1863.html) (Whonamedit.com )