Behçet disease

This is part of Rare diseases.

Diagnosis: Behçet disease

Synonyms: --


Date of publication: 2014-10-09
Version: 2.0



The disease

Behçet disease is a vascular disease in which symptoms are caused by inflammation of the blood vessels. (Vasculitis, vas= vessel, itis= inflammation). The disease is characterized by recurrent painful oral ulcers and genital lesions, accompanied in many cases by inflammation of the eyes and the bowels. The disease is associated with an increased risk of thrombosis (blood clots). Many vascular disorders are autoimmune diseases, in which the immune system mistakenly attacks the body’s own tissues.

Behçet disease has been known since antiquity but is named after the Turkish dermatologist Hulusi Behçet, who described the combination of skin problems and eye inflammation in 1937. Other unusual forms of vasculitis include Takayasus arteritis, Churg-Strauss syndrome and polyarteritis nodosa. Separate information on these disorders is available in the rare disease database of the Swedish National Board of Health and Welfare.


It is estimated that approximately five people per 100,000 in Sweden have the disease. In Sweden it is considerably more common in people whose origins lie in Turkey, Iraq, Iran and Afghanistan. In these countries, the eastern Mediterranean region and Japan, the disease is found much more frequently. The condition is equally prevalent in men and women.


The cause of the disease is unknown. The symptoms arise as a consequence of inflammation in the walls of the blood vessels, which in turn is caused by an autoimmune reaction. The oxygen-rich blood flow is reduced or blocked completely, leading to tissue damage. It is not known what triggers the reaction, but genetic factors as well as bacterial and viral infections are possible causes. In Behçet disease, certain changes (polymorphisms) are common in the genes which control the formation of proteins associated with the immune system. There are also indications that levels of certain transmitters produced by the immune system (cytokines), IL-17 and IL-23, are elevated. This shifts the balance between two different types of lymphocytes (types of white blood cells), Th17 cells and regulatory T cells. An increased number of Th17 cells promotes autoimmunity.

The increased risk of blood clots (thrombi) associated with the disease is caused by the elevated levels of proteins which are activated when blood coagulates.

The geographical range of the disease is similar to that of another rare disorder, familial Mediterranean fever, and the medical literature describes some families in which several members also have Behçet disease. The question has been raised whether there is a connection, but the genetic mutations that cause familial Mediterranean fever have not been found in individuals with Behçet disease.


There is no indication that the condition is inherited. Behçet disease is rarely present in several members of a family, although the risk of developing the disease is associated with a certain genetic disposition. It is likely that the underlying cause of the disease is a combination of genetic and environmental factors, although these factors have not yet been identified. Many people with the disease express the protein HLA-B51 in their cells. HLA proteins play an important role in the immune system.


The age of onset is normally between 25 and 35 but people of all ages, including children, can develop this disease. Early signs, preceding the actual onset by months or even years, include weight loss, a general feeling of illness, and headaches. There may also be recurrent throat infections.

Behçet disease can affect all types and sizes of blood vessels, and consequently may affect each person differently. However, practically all people with the disease have painful mouth sores and genital lesions. Inflammation of the eyes and skin and inflammatory bowel disease are also common. The first symptom of the condition is often the appearance of mouth ulcerations (known as aphthous ulcerations). They may resemble common cold sores (oral herpes). The symptoms are often intermittent and may persist for long periods or last no longer than a couple of weeks. The severity of the disease varies. It can confine itself to mouth ulcers, or result in severe vision impairment. It may also cause inflammation of the brain or the brain’s protective layers. As many organs can be affected, the symptoms may vary from episode to episode.

Recurrent aphthous ulcers

Everyone with this disease develops oral ulcers, similar in appearance to the condition known as aphthous stomatitis. These ulcers typically appear on the lips, tongue and on the inner surface of the cheeks, singly or in clusters. They can be very painful, causing difficulties in eating. Usually they disappear after 10 to 14 days, but often recur within weeks or months. Small sores usually heal without scarring, but larger sores may leave scars.

About half of all individuals with the disease also have genital lesions. They resemble the oral ulcers and may be deep and painful. In women they often develop on the external genitalia (the vulva). In men they appear on the scrotum and sometimes on the penis. There may also be inflammation of the epididymis.

Skin symptoms

Various skin problems often develop. Skin redness is common, as well as tender lesions that look like raised bumps (nodules) and pus-filled blisters (pustules), especially on the legs and upper torso. An acne-like rash may occur anywhere on the body. Erythema nodosum is an inflammatory reaction that causes red, tender nodules on the legs and ankles and occasionally on the face, neck and arms.

A special phenomenon is pathergy, a non-specific hypersensitivity in the skin that causes a reaction in the form of a small, pus-filled blister in response to minor trauma, such as a needle prick. Pathergy is characteristic of Behçet disease, but the phenomenon is not present in all people with the condition.

Eye Inflammation

Recurrent eye inflammation (uveitis) occurs in more than half of all people with the disease, but is more common in men than in women. Symptoms include pain, blurred vision, sensitivity to light, and red, watery eyes. If the retina is affected by vascular inflammation, vision impairment may result. Usually both eyes are affected. Eye inflammation usually develops within three years after the first symptoms have appeared. With current treatment methods, eye inflammation does not necessarily lead to visual impairment, something that used to be common. A characteristic feature of Behçet disease is the development of pus in the anterior chamber of the eye (hypopyon), between the cornea and iris. The accumulation collects in the lower part of the anterior chamber and is visible through the cornea.


Arthritis with joint pain is very common, occurring in about two-thirds of all people with the disease. The large joints are most likely to be affected, resulting in pain, swelling and stiffness. These symptoms typically last for a few weeks and then get better on their own. They very rarely damage the joint.

Vascular inflammation (vasculitis) and blood clots (thrombosis)

Behçet disease is a form of vasculitis. Most symptoms are caused by inflammation of the small arteries (blood vessels that carry oxygen-rich blood), and by blood clots. All blood vessels can be affected, including the capillaries in the kidneys known as the glomerulus, and blood vessels in the eye and brain. A small number of people with the disease are affected by inflammation and clot formation (thrombophlebitis) in the superficial veins (blood vessels that carry deoxygenated blood to the heart), usually in the legs. Others may develop blood clots in the deep veins. A few may also develop aortic aneurysms (a dilation of the aorta), which can become life-threatening if they rupture.

Neurological symptoms

The inflammation often affects the peripheral nerves (polyneuropathy), which can cause numbness and tingling. Other neurological symptoms may develop, such as difficulties in coordinating movements (ataxia) or speech disorders. The disease can sometimes cause inflammation of the brain or meninges (meningoencephalitis). Symptoms include fever, headache and stiff neck and can also affect consciousness levels. In severe cases, blood clots may form in the blood vessels of the brain. These blood clots cause strokes, the resulting symptoms depending on the blood vessel affected.

Inflammatory bowel disease

More rarely, inflammation and ulcers develop in the gastrointestinal tract, which can lead to stomach pain, diarrhoea and/or bleeding. These symptoms can be confused with those of chronic inflammatory bowel disease, particularly Crohn disease.

Other symptoms

Apart from affecting multiple organs in the body, the disease is associated with malaise, fever, fatigue, and weight loss.
The disease is very rare in children. Recurrent episodes of fever are common in children with the disease, but otherwise the symptoms are the same as in adults.

Pregnancy often results in a temporary improvement of the disease, although the condition of a small group of pregnant women with the syndrome deteriorates.


It usually takes a long time before the diagnosis can be made. There are no specific laboratory tests for identifying the disease, and the diagnosis is based on clinical criteria. There are many diagnostic classifications but the criteria adopted by the International Study Group for Behcet’s disease in 1990 and subsequently updated, are currently the most widely-used.

These criteria include recurrent ulcers in the mouth, eye inflammation and lesions on or around the genitals. To establish the diagnosis a number of other disorders must be excluded. There are many disorders associated with fever and joint pain. The skin symptoms are non-specific, and the oral ulcers may be mistaken for herpes blisters. This is one reason why other diseases must be excluded before the diagnosis can be established.


There is no cure for Behçet disease, but symptoms can be eased and it is usually possible to control the disease by medication. It is usually possible to prevent permanent damage. Treatment depends on which organs are involved and requires the collaboration of several specialists. The goal is to relieve symptoms, reduce inflammation and prevent organ damage.

A combination of drugs is often required. They may have a local effect on the skin or mucous membranes or a systemic effect, where tablets or injections affect the whole body. In order to reduce inflammation in the skin and mucous membranes, cortisone creams (corticosteroids) can be used. Other anti-inflammatory drugs or anaesthetic creams may also be used to ease pain.

Systemic treatment

Cortisone in tablet form is often used, particularly in the early stages of the disease where episodes of acute deterioration occur, or when organs such as the eyes, intestines or nervous system are affected. Long-term use results in a characteristic redistribution of body fat combined with weight gain, mood changes and an increased risk of osteoporosis.

Treatment with colchicine, a drug that has long been used to treat gout, can alleviate skin and joint symptoms. Common side effects include nausea, abdominal pain and diarrhoea, symptoms that may disappear by avoiding dairy products for a few weeks. Sometimes the side effects become severe, in which case the colchicine dose has to be reduced or treatment halted. Immunosuppressive medication of the same type as used in transplantation (calcineurin inhibitors and azathioprine) can be used where possible to reduce the corticosteroid dose. These medications can lead to fewer relapses and reduce inflammation throughout the body, and are used particularly for inflammation of the eye and the central nervous system.

Chemotherapy in the form of cyclophosphamide may also be used.
Biological medication is now frequently used. This type of treatment can be used to inhibit different signalling pathways in the immune system. One example of such a pathway is TNF, tumour necrosis factor, which is a substance (a cytokine) that promotes inflammation and is produced by immune system cells. In many cases, it is possible to achieve a good effect on the disease by inhibiting the binding of TNF to its receptor on different cells.

Thalidomide (earlier known in Sweden as Neurosidyn) is used to treat resistant mouth ulcers and genital lesions. Because this substance causes severe birth defects its use requires a special license. Owing to the high risk of birth defects, women of childbearing age who are being treated with thalidomide must use two approved methods of contraception, and men who are being treated must use a condom during intercourse as thalidomide may be found in sperm. Treatment with thalidomide should be stopped at least four weeks before a planned pregnancy.

If these treatments are insufficient, or if the disease is severe, many other medications can be tried. Some affected organs may require specific treatment. Cortisone can for example be used in eye drops, or in cases of very active eye disease it can be injected into the eye. Recurrent blood clots should be treated with blood-thinning medicines, and chemotherapy is used to slow underlying inflammation. Bowel disease may require similar treatment to that of chronic inflammatory bowel disease.

Most people with Behçet disease can live relatively active lives with the help of medication. When the treatment is working well relapses are less frequent. In many people symptoms diminish with age.

Social and psychological support is important. The disease is often associated with tiredness and lack of initiative, while cortisone treatment affects the mood and sometimes causes depression and sleeping problems. It is important that people close to the person with the disease understand how severe the social and psychological consequences of Behçet disease can be.

Practical advice


National and regional resources in Sweden

Paediatric Immunology, Department of Medicine, The Queen Silvia Children’s Hospital, SE-416 85 Gothenburg, Sweden. Tel: +46 31 343 40 00.

The Rheumatology Clinic, Karolinska University Hospital, Huddinge, SE-141 86 Stockholm, Sweden. Tel: +46 8 585 800 00.

Resource personnel

Associate Professor Johan Bratt, Rheumatology Clinic, Karolinska University Hospital, Huddinge, SE-141 86 Stockholm, Sweden. Tel: +46 8 585 800 00, email: johan.bratt@karolinska.se.

Professor Anders Fasth, The Queen Silvia Children’s Hospital, SE-416 85 Gothenburg, Sweden. Tel: +46 31 343 40 00, email: anders.fasth@gu.se.

Courses, exchanges of experience, recreation


Organizations for the disabled/patient associations etc.  

The Swedish Rheumatism Association, Alströmergatan 39, Stockholm. Mailing address: Box 12851, SE-112 98 Stockholm, Sweden. Tel: +46 8 505 805 00, fax: +46 8 505 805 50, email: info@reumatikerforbundet.org, www.reumatikerforbundet.org.

Neuro Sweden, Box 49084, St Eriksgatan 44, SE-100 28 Stockholm, Sweden. Tel: +46 8 677 70 10, email: info@neuroforbundet.se, www.neuroforbundet.se.

SRF, The Swedish Association of the Visually Impaired, Sandsborgsvägen 52, SE-122 88 Enskede, Sweden. Tel: +46 8 39 90 00, fax: +46 8 39 93 22, email: info@srf.nu, www.srf.nu.

Courses, exchanges of experience for personnel


Research and development

Because of the rarity of the disease in Sweden there is currently no research under way here. In countries where the disease is more common, including Turkey, Iran and China, studies are ongoing. Contact: Professor Seza Ozen, Hacettepe University, Ankara, Turkiet (sezaozen@hacettepe.edu.tr).

Information material

Short summaries of all the database texts are available as leaflets, in Swedish only. These leaflets may be ordered or printed out. (See under “Mer hos oss” in the right hand column.)


Azizlerli G, Akdag Kose A, Sarica R, E Saglik, Azizlerli G. Prevalence of Behcet’s disease in Istanbul, Turkey. Int J Dermatol 2003; 42: 803-806.

Behcet H. Über rezidivierende, aphthose, durch ein Virus verursachte Geshwure am Munde, am Auge und an den Genitalien. Dermatologische Wochenschrift 1937; 36: 1152-1157.

Dursun A, Durakbasi-Dursun HG, Zamani AG, Gulbahar ZG, Dursun R, Yakicier C. Genetic analysis of MEFV gene pyrin domain in patients with Behcet’s disease. Mediat Inflamm 2006; 2006: 41783.

Estrach C, Mpofu S, Moots RJ. Behcet’s syndrome: response to infliximab after failure of etanercept. Rheumatology (Oxford) 2002; 41: 1213-1214.

Fernández-Bello I, López-Longo FJ, Arias-Salgado EG, Jiménez-Yuste V, Butta NV. Behçet’s disease: new insight into the relationship between procoagulant state, endothelial activation/damage and disease activity. Orphanet J Rare Dis. 2013; 8: 81.

Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A et al. Management of Behçet disease: a systematic literature review for the European League Against Rheumatism evidence-based recommendations for the management of Behçet disease. Ann Rheum Dis 2009; 68: 1528-1534.

Idil A, Gurler A, Boyvat A, Caliskan D, Ozdemir O, Isik A et al. The prevalence of Behcet’s disease above the age of 10 years. The results of a pilot study conducted at the Park Primary Health Care Center in Ankara, Turkey. Ophthalm Epidemiol 2002; 9: 325-331.

International Study Group for Behcet’s Disease, Criteria for diagnosis of Behcet’s disease. International Study Group for Behcet’s Disease. Lancet 1990; 335: 1078-1080.

Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 2013; 65: 1-11.

Kari JA, Shah V & Dillon MJ. Behcet’s disease in UK children: clinical features and treatment including thalidomide. Rheumatology (Oxford) 2001; 40: 933-938.

Kim ES, Kim SW, Moon CM, Park JJ, Kim TI, Kim WH et al. Interactions between IL17A, IL23R, and STAT4 polymorphisms confer susceptibility to intestinal Behcet’s disease in Korean population. Life Sci. 2012; 90: 740-6.

Kirino Y, Zhou Q, Ishigatsubo Y, Mizuki N, Tugal-Tutkun I, Seyahi E et al. Targeted resequencing implicates the familial Mediterranean fever gene MEFV and the toll-like receptor 4 gene TLR4 in Behçet disease. Proc Natl Acad Sci U S A. 2013; 110: 8134-9.

Kone-Paut I, Yurdakul S, Bahabri SA, Shafae N, Ozen S, Ozdogan H et al. Clinical features of Behcet’s disease in children: an international collaborative study of 86 cases. J Pediatr 1998; 132: 721-725.

Mendoza-Pinto C, García-Carrasco M, Jiménez-Hernández M, Jiménez Hernández C, Riebeling-Navarro C, Nava Zavala A et al. Etiopathogenesis of Behcet’s disease. Autoimmun Rev 2010; 9: 241-245.

Mohammad A, Mandl T, Sturfelt G, Segelmark M. Incidence, prevalence and clinical characteristics of Behcet’s disease in southern Sweden. Rheumatology (Oxford). 2013; 52: 304-10.

Ozen S, Eroglu FK. Pediatric-onset Behçet disease. Curr Opin Rheumatol. 2013; 25: 636-42.

Saadoun D, Wechsler B. Behçet’s disease. Orphanet J Rare Dis. 2012; 7: 20.

Saleh Z, Arayssi T. Update on the therapy of Behçet disease. Ther Adv Chronic Dis. 2014;5:112-134.

Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN. Effect of infliximab on sight-threatening panuveitis in Behcet’s disease. Lancet 2001; 358: 295-296.

Database references

OMIM (Online Mendelian Inheritance in Man)
Search: Behcet syndrome, Behçet disease

Document information

The Swedish Information Centre for Rare Diseases produced and edited this information material.

The medical expert who wrote the draft of this information material is Professor Anders Fasth, The Queen Silvia Children’s Hospital, Gothenburg, Sweden.

The relevant organisations for the disabled/patient associations have been given the opportunity to comment on the content of the text.

An expert group on rare diseases, affiliated with the University of Gothenburg, approved the material prior to publication.

Date of publication: 2014-10-09
Version: 2.0
Publication date of the Swedish version: 2014-06-23

For enquiries contact The Swedish Information Centre for Rare Diseases, The Sahlgrenska Academy at the University of Gothenburg, Box 422, SE-405 30 Gothenburg, Sweden. Tel: +46 31 786 55 90, email: ovanligadiagnoser@gu.se.


About the database

This knowledge database provides information on rare diseases and conditions. The information is not intended to be a substitute for professional medical care, nor is it intended to be used as a basis for diagnosis or treatment.