Behcets Disease

Information and facts about Eponymous diseases.

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.

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.

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.

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.

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.

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.

Back to Eponymous diseases index page

Can't find what you are looking for?
Use our site search facility...

Home I Search Site I Site map I Conditions I Symptoms I News Archive I Forum I Contact us I About us I Access Keys

All information published on this web site is for information purposes only. The content of this web site should not be used for a conclusive diagnosis or for choosing a treatment. The content on this site has been provided as a guideline and general information it is not intended to replace professional medical care. In all serious cases it is advisable to recieve attention from a qualified medical practitioner. All text is available under the terms of the GNU free documentation license.