What Is Behçet's Disease? What Are Its Symptoms and Treatment?

Behçet's disease is a chronic, systemic health problem that involves inflammation of the blood vessels in the body and manifests itself through mouth ulcers (aphthae), genital sores, and involvement of the eyes, skin, joints and internal organs. At the root of the disease lies inflammation that develops in the walls of the blood vessels. Because both small and large vessels can be affected, the resulting symptoms can vary considerably. Behçet's disease is considered a rare disease. It is seen more frequently in certain geographical regions, including Türkiye. Although it usually appears in young adulthood, there are also cases of Behçet's disease in children and cases seen at older ages. In the article below, you can find answers to frequently asked questions such as "What Is Behçet's Disease?", "What Causes Behçet's Disease?" and "Is Behçet's Disease Contagious?", and much more.

What Is Behçet's Disease?

Behçet's disease is a chronic, inflammatory disease that can affect the blood vessels in the body. The disease may not remain limited to a single area. Although mouth ulcers and sores in the genital area are among the best-known signs of Behçet's disease, the eyes, skin, joints, digestive system, blood vessels and nervous system can also be affected. For this reason, Behçet's disease does not present in the same way in every person. In some people mouth ulcers are the main feature, while in others eye findings, vascular involvement or joint complaints may be more prominent.
In Behçet's disease, symptoms may appear periodically. A new flare may follow calm periods in which complaints subside. While some findings may regress within a few weeks, another organ system may be affected at a different stage of the disease. This variable course can make it difficult to base the diagnostic process on a single symptom alone.

What Are the Symptoms of Behçet's Disease?

The signs of Behçet's disease and its symptoms can vary from person to person. One of the most common findings is recurrent mouth ulcers. These sores may start as small raised bumps and quickly turn into painful ulcers. The sores usually heal within a few weeks, but they may reappear at certain intervals. Some other symptoms of Behçet's disease are as follows:

• Genital involvement in Behçet's disease causes painful sores, which are also among the important findings. In women, they can appear around the vulva in the form of vaginal sores in Behçet's disease, and in men in the scrotal area. In some people, the sores may leave scars after healing. Not every sore in the genital area means Behçet's disease. For this reason, recurrent or unexplained genital sores require evaluation by a physician.
• Skin findings and skin lesions in Behçet's disease can appear in different forms. Acne-like lesions or painful, raised nodules, particularly on the lower legs, may develop. Eye involvement in Behçet's disease, on the other hand, can cause complaints such as redness of the eye, pain, sensitivity to light and blurred vision. Because untreated inflammation inside the eye can lead to serious consequences such as vision loss, eye symptoms should be taken seriously.
• Pain and swelling in the joints may also occur. The knees are among the most frequently affected joints. Complaints may also arise in areas such as the ankles, elbows and wrists. When there is vascular involvement, pain and swelling may be seen, particularly in the arms or legs. Involvement of the digestive system may manifest as abdominal pain, diarrhea or bleeding in the digestive tract. Involvement of the nervous system, on the other hand, can cause headache, fever, changes in consciousness, balance problems or more serious neurological findings.

What Are the First Symptoms of Behçet's Disease?

Recurrent mouth ulcers stand out among the first symptoms. These sores may resemble ordinary canker sores, but their tendency to recur is important for diagnostic evaluation. Recurrent sores in the genital area or unexplained skin lesions may also be seen in the early period. In some people, the first complaint may be redness, pain or blurred vision in the eye. In some people, joint pain or painful skin nodules on the legs may be more prominent. For this reason, all symptoms are not expected to appear at the same time at the onset of the disease. A mouth ulcer alone or a single skin lesion is not sufficient for a diagnosis of Behçet's disease. In particular, the course of recurrent symptoms over time is important for diagnosis.

What Causes Behçet's Disease?

The exact cause of Behçet's disease is not fully known. Genetic predisposition and environmental factors are thought to play a role together in the development of the disease. Changes in the way the immune system works may contribute to inflammation of the blood vessels. However, it is not possible to attribute the disease to a single cause. Certain genetic characteristics are known to increase susceptibility to Behçet's disease. One of the genetic markers, called HLA-B51, is also associated with this susceptibility. However, the presence of this marker alone does not establish a diagnosis of Behçet's disease. The disease is not expected to develop in every person who has the same genetic characteristic. Similarly, not every person with Behçet's disease necessarily has this marker.
The fact that the disease is seen more frequently in Türkiye, the Middle East and some East Asian countries has led researchers to investigate environmental factors in addition to genetic predisposition. However, no particular food, stressful period or any single environmental factor can be shown to be the definitive cause of Behçet's disease.

What Is Good for Behçet's Disease?

In Behçet's disease, it is not expected that all symptoms can be eliminated with a single method. Treatment is planned according to which organs the disease affects and the severity of the symptoms. The goal is to keep the inflammation under control, reduce symptoms and prevent permanent damage to important organs such as the eyes, blood vessels or nervous system. Patients looking for foods that are good for Behçet's disease should know that nutrition alone will not be sufficient. For mouth ulcers, oral care products and topical treatments aimed at reducing pain can be used. For skin findings, creams or gels recommended by the doctor can be used. If there is eye involvement, appropriate eye treatments are applied according to the condition of the eye. For joint complaints, drug treatment can also be planned according to the overall state of the disease. Adequate rest and regular sleep may be important in daily life. Reducing excessive physical and psychological strain during flares may be beneficial. Moderate physical activities such as walking or swimming, in line with the course of the disease, may also contribute to general well-being. However, no food or herbal product should be thought to cure Behçet's disease. In particular, it is not appropriate to take supplements that may interact with the medications being used without consulting a doctor.

What Is the Treatment for Behçet's Disease?

The treatment of Behçet's disease varies according to the organs affected by the disease. Topical treatments can be used for mild oral or genital sores. In some patients, Behçet's disease medications such as colchicine or apremilast may be preferred to control oral and genital ulcers. In cases with more pronounced inflammation or organ involvement, medications that reduce the activity of the immune system may be needed. New treatment methods for Behçet's disease developed today include approaches that target the immune response. In severe cases, biologic drugs may also be among the treatment options. However, the use of these drugs is decided by a specialist physician after evaluating the characteristics of the disease and the person's general health. Not every Behçet's patient receives the same drug or the same dose.
Regular follow-up makes up an important part of treatment. Because Behçet's disease can affect more than one organ, evaluation by physicians from different specialties may be necessary. In particular, evaluation should not be delayed when new complaints arise such as decreased vision, significant eye pain, sudden neurological symptoms or signs suggesting vascular involvement. It is also not correct to stop medications on one's own when symptoms subside. Even if the disease appears to have calmed down, treatment should not be changed or discontinued without the physician's decision. Regular check-ups help detect a new flare of the disease and organ damage early.

Which Organ Does Behçet's Disease Most Commonly Affect?

One of the most common findings in Behçet's disease is recurrent sores inside the mouth. In addition, the genital area and the skin are among the frequently affected areas. Eye involvement is also one of the important findings of the disease. However, Behçet's disease should not be regarded only as a disease of the mouth or skin. Because the disease affects the blood vessels, vascular involvement is of particular importance. Involvement of the veins or arteries can cause different problems. Involvement of large vessels can lead to serious complications in some cases. Eye and nervous system involvement are also conditions that must be closely monitored because of the risk of permanent damage. Therefore, the answer to the question of which area is most commonly affected cannot be limited to a single organ. Although mouth ulcers are one of the most characteristic and common symptoms of the disease, it should not be forgotten that the disease is systemic in nature.

What Is a Behçet's Disease Attack Like?

Behçet's disease attack is the term used for periods that are generally considered to be flares of the disease. During these periods, previously seen symptoms may reappear or new organ involvement may develop. For example, the following symptoms may be seen at the same time or at different times:

• Recurrent mouth ulcers
• Genital sores
• Skin lesions
• Joint pain
• Eye complaints

The severity of a flare varies from person to person. At some times only mouth ulcers are seen, while in other cases the eyes, blood vessels or nervous system may be affected. In particular, symptoms such as changes in vision, severe eye pain, sudden weakness, speech disturbance, marked headache or sudden swelling in the arm or leg may suggest more serious involvement. During a flare, it is important to rest, reduce stress and continue the current treatment as recommended by the doctor. When a new or unusual symptom appears, it may be necessary to go to a healthcare facility without waiting for the follow-up appointment.

What Should Not Be Eaten With Behçet's Disease?

In Behçet's disease, there is no definitive list of forbidden foods that applies to all patients. What should not be eaten in Behçet's disease may differ from person to person. Treatment of the disease is not based on completely eliminating a particular food group. During periods when mouth ulcers are active, very spicy, sour or other foods that irritate the oral mucosa may increase pain. During such periods, softer and non-irritating foods may be preferred according to personal tolerance. An adequate and balanced diet is important for general health. A monotonous diet or unnecessary restrictions should be avoided. If it is noticed that complaints consistently increase after eating a particular food, it is better to note this and discuss it with a doctor or dietitian. Special diets or a single food claimed to cure Behçet's disease should not be relied upon. Nutrition does not replace the treatment of the disease. Medications should be taken regularly and medical follow-up should be continued.

How Is Behçet's Disease Diagnosed?

Behçet's disease is not diagnosed with a single blood test or with imaging alone. Although there is no single test that directly detects Behçet's disease, it is important to evaluate the medical history, physical examination and current symptoms together during the diagnostic process. Taking the diagnostic criteria for Behçet's disease into account, the co-occurrence of recurrent mouth ulcers with genital sores, skin findings or eye inflammation is examined in particular. Blood tests can help rule out other diseases or assess inflammation in the body. Some genetic markers such as HLA-B51 can provide supporting information, but they do not establish a diagnosis on their own. The history of symptoms is particularly important in the diagnostic process. How often the mouth ulcers recur, whether there are sores in the genital area, whether there have been eye complaints and whether skin or joint findings accompany them are evaluated. When necessary, opinions may be obtained from different specialties such as ophthalmology, neurology, dermatology or gastroenterology.

Is Behçet's Disease Genetic?

Genetic predisposition plays a role in Behçet's disease, but the disease is not considered a simple hereditary disease transmitted directly through a single gene. In addition to genetic characteristics, different factors such as the way the immune system works and environmental influences are thought to play a role together in the development of the disease. Therefore, the presence of a particular genetic characteristic alone does not mean that the disease will develop. The association between HLA-B51 and Behçet's disease can be given as an example of genetic predisposition. HLA-B51 refers to a particular immune system characteristic and may be seen more often in people with Behçet's disease than in the general population. However, being an HLA-B51 carrier does not establish a diagnosis of the disease. Behçet's disease does not develop in everyone who carries this genetic characteristic. Likewise, not every person with Behçet's disease is expected to have HLA-B51. Genetic predisposition is regarded more as one of the factors that increase susceptibility to the disease. In other words, genetic characteristics alone may not be sufficient for the disease to appear. Other factors, such as the response of the immune system and environmental conditions, are also thought to contribute to the process. For this reason, the development of Behçet's disease cannot be attributed to a single cause.
The presence of Behçet's disease in the family should be evaluated in a similar way. The occurrence of the disease in first-degree relatives may suggest that there is a genetic predisposition, but it does not mean that Behçet's disease will necessarily develop in other family members. Family history is one of the pieces of information that can be taken into account when assessing disease risk, but it does not establish a diagnosis on its own. The pattern of genetic transmission of Behçet's disease differs from that of many classic hereditary diseases. The disease is not thought to arise from the transmission of a particular gene alone. Therefore, if there is Behçet's disease in the family, it is not correct to conclude that the disease will definitely be passed on to future generations.
Just as having a genetic predisposition does not mean that the disease will definitely develop, the absence of genetic risk factors does not completely rule out the disease. In the diagnostic process, the signs and symptoms caused by the disease and the clinical evaluation are more important than genetic characteristics. A diagnostic workup can be carried out by evaluating symptoms such as recurrent oral and genital sores, eye findings, skin lesions and vascular or nervous system involvement.

Which Department Follows Up Behçet's Disease?

The rheumatology department plays an important role in the follow-up of Behçet's disease. Because the disease can affect more than one organ system, the rheumatologist can evaluate the overall course of the disease and plan treatment according to organ involvement. Depending on the symptoms, evaluation by other specialties may also be necessary. If there is redness, pain or a change in vision in the eye, it is important to be examined by an ophthalmologist. When skin findings are prominent, a dermatology evaluation may be necessary. When digestive system complaints or neurological symptoms arise, support can be obtained from the relevant specialties.

Which Doctor Treats Behçet's Disease?

For Behçet's disease, a rheumatologist can be consulted first. The rheumatologist can evaluate the systemic features of the disease, plan the necessary tests and follow up the treatment. In people with eye involvement, evaluation by an ophthalmologist is particularly important. Behçet's disease can cause inflammation in the eye, so an examination should be carried out without delay when a new symptom related to vision appears. For symptoms that appear in childhood, a pediatric rheumatology evaluation may be necessary. It is common for different specialties to carry out follow-up together, depending on the organ affected by the disease.

Is Behçet's Disease Contagious?

Behçet's disease is not contagious. It is not expected to pass from one person to another through contact. The presence of sores in the mouth or genital area does not mean that the disease is contagious. Underlying the disease are mechanisms related to genetic predisposition and the way the immune system works. For this reason, Behçet's disease is not considered a disease that is transmitted from person to person, as is the case with infectious diseases. Because of the presence of sores in the genital area, the disease should not be thought of as a sexually transmitted disease. At the same time, it should not be forgotten that not every sore that appears in the genital area is Behçet's disease. Recurrent genital sores, or those whose cause cannot be determined, require appropriate medical evaluation.

Is Behçet's Disease Fatal?

Behçet's disease does not follow the same course with the same severity in every person. In many people, the symptoms can be brought under control and life can be maintained with regular follow-up. However, some forms of organ involvement can lead to serious complications. Close follow-up is required, especially when the blood vessels, eyes or nervous system are affected. Untreated inflammation in the eye can progress to vision loss. Vascular involvement, on the other hand, can cause complications such as clotting or serious changes in the vessel wall. Nervous system involvement can also have significant consequences. For this reason, Behçet's disease should not be taken lightly. Whether the disease is serious cannot be determined by looking at the name of the diagnosis alone. Which organs are affected and how active the disease is must be evaluated.

Does Behçet's Disease Affect Sexual Life?

Behçet's disease can affect sexual life, especially during periods when there are active sores in the genital area. Because genital ulcers can be painful, discomfort or pain may be experienced during sexual intercourse. These complaints may decrease as the sores heal. It is also possible for some sores to leave scars after healing. The effects on sexual life may not be limited to physical pain. Anxiety or avoidance of sexual intercourse may develop because of recurrent sores. This does not mean that the disease directly causes a loss of sexual function. Continuing sexual intercourse despite pain during a period of active genital sores may increase the discomfort in the area. If the complaints recur or significantly affect sexual life, it is advisable to talk openly with the relevant physician.

Does Behçet's Disease Cause Forgetfulness?

Nervous system involvement can be seen in Behçet's disease. When the nervous system is affected, headache, changes in consciousness, balance problems or other neurological symptoms may appear. Forgetfulness is not one of the most typical symptoms of Behçet's disease. However, a neurological evaluation may be necessary if newly developing, marked cognitive changes are accompanied by other symptoms such as headache, impaired balance, changes in vision, weakness or altered consciousness. Forgetfulness in daily life can have many different causes. Irregular sleep, stress, the medications being used and other health problems can also cause cognitive complaints. For this reason, not every instance of forgetfulness should be attributed to Behçet's disease.

What Disability Rating Is Given for Behçet's Disease?

For Behçet's disease, there is no single, fixed disability rating that can be applied to all patients. The assessment may vary according to which organs the disease affects and the extent to which this involvement causes a loss of capacity in daily life and in the ability to work. Limited complaints such as mouth ulcers and serious eye, vascular or nervous system involvement are not evaluated in the same way. Whether there is permanent organ damage may also be important in the assessment. To evaluate disability status or the need for a medical board report, an examination must be carried out at an authorized healthcare institution in accordance with the relevant legislation. It is not correct to determine the rating in advance based solely on the diagnosis of Behçet's disease. The person's current health status and documented organ involvement are taken as the basis.

Does Behçet's Disease Cause Itching?

Itching is not one of the main and most characteristic symptoms of Behçet's disease. Nevertheless, skin involvement can appear in different forms. Acne-like lesions or painful, raised nodules, especially on the legs, may be seen. The presence of itching alone does not suggest Behçet's disease. Allergic reactions, skin diseases, infections, drug reactions and many other conditions can cause itching.
In a person diagnosed with Behçet's disease, it should not be assumed that newly developing or spreading itching is directly related to the disease. If new lesions develop on the skin, it is advisable to have them evaluated by a dermatologist or by the physician who carries out the general follow-up of the disease.
Behçet's disease is a systemic disease that may require long-term follow-up. Although recurrent sores in the mouth and genital area are among the best-known signs of the disease, the disease can present a much broader picture. Inflammation in the eyes, vascular involvement, joint complaints, skin findings, digestive system problems and neurological symptoms may appear at different times.
Because there is no single test for diagnosis, the history of symptoms and their course over time are important. During the physician's evaluation, providing detailed information about previous mouth ulcers, genital ulcers, eye complaints, skin lesions and joint problems can help the diagnostic process.
Although expressions such as "people who have recovered from Behçet's disease" are used among the public, from a medical point of view it is more accurate to speak of the disease being kept under control and of a period of remission. The disease may calm down from time to time and may not cause symptoms for a long period. Even so, it is not appropriate to stop follow-up. Regular medical check-ups are particularly important if there has previously been involvement of the eyes, blood vessels or nervous system.
When newly developing blurred vision, eye pain, sudden neurological changes, marked swelling of the arm or leg, severe headache or other serious symptoms appear, medical evaluation should not be delayed. With appropriate treatment and regular follow-up, it may be possible to prevent serious complications that the disease can cause. You too can benefit from appropriate follow-up and treatment methods by making an appointment at Yeditepe University Hospitals.