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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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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".


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.


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)


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Behcet disease, Adamantiades-Behcet disease, or Hippocrates-Adamantiades-Behcet disease? - Letter to the Editor
From CHEST, 7/1/02

To the Editor:

Tirilomis (December 2001) (1) was correct in stating that the first description in contemporary times of Behcet disease was made by the Greek ophthalmologist Benedict Adamantiades in 1931, and that the disease was described for the second time, independently, by the Turkish dermatologist Hulusi Behcet in 1937. Actually, according to Kaklamani et al, (2) before publishing in 1931, Adamantiades in 1930 orally presented at the Medical Society of Athens (3) the case of a 20-year-old man who had developed recurrent hypopyon iritis resulting in blindness, phlebitis, oral and genital ulcerations, and knee arthritis. One year later, he published this case in the journal Annales d'Oculistique, as was stated by Tirilomis. (1)

Historically speaking, the first description of the symptomatology of this disease was reported by Hippocrates in 500 BC. (4) Therefore, to be historically correct, the complete eponym for this disease should be Hippocrates-Adamantiades-Behcet disease. However, most authors refer to it as Behcet disease, because this is the name that is in use worldwide. A recent MEDLINE search by this author yielded 3,247 references to Behcet disease in contrast to only 31 references to Adamantiades-Behcet disease.

Correspondence to: Tsung O. Cheng, MD, FCCP, Professor of Medicine, The George Washington University Medical Center, 2150 Pennsylvania Ave NW, Washington, DC 20037


(1.) Tirilomis T. Some more historical notes on Adamantiades-Behcet's disease [letter]. Chest 2001; 120:2116

(2.) Kaldamani VG, Vaiopoulos G, Kaklamanis PG. Behcet disease. Semin Arthritis Rheum 1998; 27:197-217

(3.) Adamantiades B. A case of recurrent hypopyon iritis. Athens, Greece: Medical Society of Athens, 1930; 586-593

(4.) Cheng TO. Some historical notes on Behcet's disease. Chest 2001; 119:667-668

To the Editor:

I appreciate Dr. Cheng's comments, (1) and I agree with his point that, historically speaking, the first description of the symptomatology of Adamantiades-Behcet disease was reported by Hippocrates in the fifth century BC. Should the complete eponym for the disease be Hippocrates-Adamantiades-Behcet disease? The more carefully we read the Hippocratic Corpus, the more often we discover the genius of Hippocrates. Symptoms of many diseases were first reported by Hippocrates. He not only carefully observed the clinical features of diseases, but he thoughtfully interpreted their pathologic and physiologic aspects, and treated diseases by natural means. He developed medicine as a science. Therefore, to be historically correct, probably many of the eponyms of diseases should be renamed and called Hippocrates- ... disease or syndrome. However, eponyms for diseases mostly have been attributed to contemporary scientists. Hippocrates has an outstanding position in medicine, but he is out of the competition for eponyms. His outstanding position is not only indicated by the importance of the Hippocratic Oath in the medical ethics of physicians all around the world, but Hippocrates is, without any contradiction, the father of medicine.

Should the disease be called only Behcet disease, because this name is in wide use? The use of an eponym for a clinical sign, medical technique, or a disease is an honor for every physician and scientist, and it is also a tribute to outstanding members of the medical community. The first description of the Silk Route disease in contemporary times was made by Benedict Adamantiades (2) and for the second time, independently, by Hulusi Behcet. (3) Even with delay, the tribute to Benedict Adamantiades should be given too. In dual commemoration of the contribution of these two excellent physicians and scientists to the syndrome, the disease is correctly called Adamantiades-Behcet disease, (4) and this name should be used universally.

Correspondence to: Theodor Tirilomis, MD, Department of Thoracic, Cardiac, and Vascular Surgery, University of Gottingen, Robert-Koch Str 40, D-37075 Gottingen, Germany


(1) Cheng TO. Some historical notes on Behcet's disease. Chest 2001; 119:667-668

(2) Adamantiades B. Sur un cas d'iritis a hypopion recidivant. Ann Ocul (Paris) 1931; 164:271-278

(3) Behcet H. Uber rezidivierende, aphthose, durch ein Virus verursachte Geschwure am Mund, am Auge und an den Genitalien. Dermat Wschr 1937; 105:1152-1157

(4) Tirilomis T. Some more historical notes on Adamantiades-Behcet's disease. Chest 2001; 120:2116

COPYRIGHT 2002 American College of Chest Physicians
COPYRIGHT 2002 Gale Group

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