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Behçet Syndrome: Causes, Symptoms & Treatment

Behçet syndrome is a rare, chronic inflammatory vascular disease primarily affecting the mouth, eyes, and skin. Early diagnosis is key to preventing serious complications.

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Behçet syndrome is a rare, chronic inflammatory vascular disease primarily affecting the mouth, eyes, and skin. Early diagnosis is key to preventing serious complications.

What is Behçet Syndrome?

Behçet syndrome (also known as Behçet disease) is a rare, systemic form of vasculitis – an inflammation of blood vessels that can affect virtually any organ system in the body. The condition follows a chronic course with flare-ups and periods of remission. It was first described in 1937 by Turkish dermatologist Hulusi Behçet. The disease is most prevalent along the ancient Silk Road, particularly in Turkey, the Middle East, and East Asia.

Causes and Risk Factors

The exact cause of Behçet syndrome remains incompletely understood. A combination of genetic, immunological, and environmental factors is thought to contribute:

Genetic predisposition: A strong association with the HLA-B51 gene has been established, particularly in populations along the Silk Road.
Immune dysregulation: Overactivation of both the innate and adaptive immune systems plays a central role in driving inflammation.
Infectious triggers: Certain bacteria (e.g., Streptococcus sanguinis) and viruses are discussed as possible environmental triggers.
Age and sex: The disease most commonly manifests between the ages of 20 and 40; men tend to have a more severe course than women.

Symptoms

Behçet syndrome is characterized by a wide range of symptoms affecting multiple areas of the body:

Classic Cardinal Symptoms

Recurrent oral ulcers: Painful, recurring aphthous sores in the mouth (present in over 95 % of cases).
Genital ulcers: Painful sores in the genital area, which often leave scars.
Ocular involvement (uveitis): Eye inflammation that can lead to blindness if left untreated.
Skin lesions: Including erythema nodosum (painful red nodules), pseudofolliculitis, and acneiform lesions.

Additional Possible Symptoms

Joint inflammation (arthritis)
Vascular involvement (thrombosis, aneurysms)
Neurological symptoms (Neuro-Behçet): headaches, cognitive disturbances, meningitis
Gastrointestinal symptoms (intestinal Behçet syndrome)

Diagnosis

There is no specific laboratory test for Behçet syndrome. Diagnosis is made clinically based on the International Classification Criteria of the International Study Group (ISG, 1990):

Recurrent oral ulcers (at least 3 times within 12 months)
Plus at least 2 of the following: genital ulcers, eye lesions, skin changes, or a positive pathergy test

The pathergy test is a skin prick test in which an exaggerated skin reaction to a needle puncture is considered positive. Imaging (MRI, CT) and laboratory tests help exclude other conditions and assess organ involvement.

Treatment

There is currently no curative treatment for Behçet syndrome. The goal of therapy is to control inflammatory flares and prevent organ damage:

Pharmacological Treatment

Corticosteroids: Used for rapid anti-inflammatory effect during acute flares.
Colchicine: Particularly effective for skin manifestations and arthritis.
Immunosuppressants: Azathioprine, methotrexate, or ciclosporin A for severe disease.
Biologics: TNF-alpha inhibitors such as infliximab or adalimumab for refractory cases; interferon-alfa for ocular involvement.
Apremilast: A newer agent approved for the treatment of oral ulcers in Behçet syndrome.

Supportive Measures

Regular ophthalmological monitoring to protect vision
Physiotherapy for joint involvement
Psychosocial support for those managing a chronic condition

Prognosis

The prognosis of Behçet syndrome is variable and depends greatly on which organs are affected. The greatest risks are associated with ocular, vascular, and neurological involvement. With early and consistent treatment, many patients can achieve a good quality of life. Disease activity may decrease over time in some individuals.

References

Yazici H, Seyahi E, Hatemi G, Yazici Y. Behçet syndrome: a contemporary view. Nature Reviews Rheumatology, 2018; 14(2): 107–119. DOI: 10.1038/nrrheum.2017.208
International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet, 1990; 335(8697): 1078–1080.
Kalra S, Silman A, Akman-Demir G, et al. Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. Journal of Neurology, 2014; 261(9): 1662–1676.

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