In 1937, the Turkish dermatologist and scientist Hulusi Behçet observed several patients visiting his clinic with general symptoms such as mouth and genital ulcers, uveitis (inflammation of the middle layer of the eye), and erythema nodosum. These observations eventually led to the identification of this syndrome, which was later named after him. In this article, we will explore this condition, its symptoms, and how to manage them.
First, what does the term syndrome mean? It is a medical term used to describe a group of symptoms and clinical signs that occur together and are characteristic of a specific disease.
Behçet’s Syndrome is an autoimmune condition of unknown exact cause that affects blood vessels, leading to their inflammation and swelling, along with general inflammatory reactions throughout the body. Patients often visit their doctor complaining of recurrent oral and genital ulcers or painful eye inflammation. Upon examination, widespread vasculitis (inflammation of the blood vessels) is often found, allowing physicians to diagnose the condition based on the combination of symptoms.
Like other autoimmune diseases, the exact cause of Behçet’s Syndrome is not fully understood, but genetic and environmental factors are believed to play a major role. It is more common among individuals from regions stretching from East Asia to the Eastern Mediterranean, including Central Asia, Iran, and Turkey.
Carrying the HLA-B51/B5 gene—which is common among Japanese, Middle Eastern, and Turkish populations—also significantly increases the risk of developing Behçet’s Disease, clearly indicating the interaction between genetic predisposition and environmental triggers in its development.
Ongoing research continues to investigate the precise causes and contributing factors of this disease. While several additional genetic and immune-related factors have been identified, their exact relationship to the onset of Behçet’s Disease remains a topic of scientific debate and study.
As mentioned earlier, the symptoms of Behçet’s Disease are often general and non-specific, making it difficult to pinpoint the underlying cause at first. Since the initial signs often include skin ulcers and eye inflammation, patients may first consult a dermatologist or an ophthalmologist, as the symptoms resemble other common conditions, this can lead to delayed diagnosis. The main symptoms of the disease include:
There is no single test that can confirm Behçet’s Syndrome. Diagnosis mainly depends on the pattern and recurrence of symptoms. However, the doctor may request certain laboratory and imaging tests to support the diagnosis and rule out similar diseases. These may include:
A diagnosis is confirmed based on clinical findings, usually if two or more characteristic symptoms are present with oral ulcers recuring more than three times per year a patient can get a definite diagnosis of BD.
There is no permanent cure for Behçet’s Disease; however, treatment primarily aims to control symptoms and reduce inflammation. Doctors often prescribe immunosuppressive medications to help calm the immune system, along with topical creams or ointments to treat skin ulcers. Corticosteroids may be used to relieve inflammation affecting various organs, while eye drops are given to reduce eye inflammation and prevent complications.
In cases where these medications fail to provide adequate relief, physicians may turn to biological treatment for Behcet Disease, which works by targeting specific immune pathways to suppress inflammation. Biologic therapy is not a single medication but a group of advanced treatments, and the choice of drug depends on the patient’s condition and the severity of the disease.
Behçet’s Disease is a chronic autoimmune disorder that causes inflammation in blood vessels of all sizes. This leads to recurrent mouth and genital ulcers, as well as inflammation affecting the eyes, joints, skin, and sometimes the nervous or digestive systems. The disease is chronic and fluctuating, meaning it alternates between periods of activity and remission. It is not considered dangerous in all cases, but in some patients, it can cause serious complications—especially when inflammation affects the eyes or major blood vessels.
Behçet’s Disease is generally not fatal, and most patients live normal lives with proper treatment and regular medical follow-up. However, in severe cases involving major blood vessels—particularly the pulmonary artery—or when there is significant inflammation of the central nervous system, the disease can become life-threatening. Such complications are rare but serious, making early diagnosis and consistent monitoring essential to prevent them.
Yes, Behçet’s Disease can affect the heart, although this is uncommon. Possible cardiac complications include pericarditis (inflammation of the membrane surrounding the heart), myocarditis (inflammation of the heart muscle), endocarditis (inflammation of the inner lining of the heart), and sometimes inflammation or aneurysm of the coronary arteries. These conditions require close monitoring and treatment with anti-inflammatory or immunosuppressive medications under the supervision of a rheumatologist or cardiologist.