Behçet syndrome: A Rare but Dangerous Disease

Behçet disease or Behçet syndrome is a medical condition which causes inflammation of blood vessels throughout the body. Any part of the body can be affected by this disease depending on the extent of inflammation of blood vessels. The mouth and genitals are more likely to be affected by this disease due to the extra sensitive skin.

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It is believed that this disease was first observed in the fifth century BC, but there is no documented account of this claim. The first documented account comes from 1924, when Turkish dermatologist Hulusi Behcet found this disease in one of his patients. Over the next six years, doctors began to realize that they were dealing with a new kind of disease. Then in 1930, Benediktos Adamantiades, a Greek ophthalmologist, dealt with a patient with inflammatory oral ulcers, arthritis, phlebitis, iritis, and genital ulcers. The origin of all these complications was same. Following extensive research, Hulusi Behçet published his findings about this disease in 1937. Behçet syndrome remains a very rare disease, therefore only limited studies are available about this illness.

Symptoms & Causes

The symptoms of this disease vary greatly from patient to patient. Some people show mild symptoms like sores on different parts of the skin but some people have more acute symptoms like complete loss of vision.

Some of the most common symptoms of this disease are:
• Soreness of skin, especially on mouth and genitals

• Swelling, stiffness, or pain in the joints
• Swelling of different parts of the eyes

Besides these symptoms, the following issues also indicate the inflammation of blood vessels but they are very rare:

• Blindness
• Inflammation in the nervous system
• Inflammation in spinal cord
• Inflammation in the blood vessels of digestive system

All these symptoms can disappear at any time and then reappear after some time. The time of appearance of these symptoms is also known as flares.

Young people are more likely to be affected by this disease, and it predominantly affects those in their 20s and 30s. Anything that can inflame blood vessels may lead to this disease – but the dilemma faced by doctors is that they are not sure what things cause inflammation of blood vessels. Some doctors suggest that contamination in the environment is the most likely cause of this disease, but as yet this remains an unproven theory. Another theory is that the main cause of this disease is genetic. People may inherit this disease from their parents. One thing that is known for sure is that this disease is not contagious.

Pathogenesis of Behçet

The pathophysiology of Behçet’s disease suggests that this disease is an autoimmune condition. It also indicates that our bodies can treat this disease without the aid of medication, as there is observable evidence that the symptoms can disappear after some time. According to current theory, the inflammation of blood vessels is probably caused by infectious triggers like heat shock proteins (HSPs). There are different classes of heat shock proteins (HSPs) including HSP-60 and HSP-65. It is found that the oral ulcers contain a high concentration of HSP-65. Neutrophils and T cells are also believed to play some role in the inflammation of the blood vessels. The pathophysiology also suggests that genetics play an important role in this disease. All these claims are just theories but they can provide some baseline to take the research one step ahead.

Diagnosis & Treatment

The diagnosis of Behçet’s disease is troublesome, because several symptoms of this disease match with the symptoms of other diseases, and symptoms don’t show in the first phase of this disease. Moreover, there is not yet a definitive test to diagnose this disease. Due to all these limitations, doctors have developed a set of rules to give a probable diagnosis of this disease:

If a patient has two of the following issues then there is high probability of the presence of Behçet’s disease:

• Inflammation of the eyes
• Soreness of genitals
• Positive pathergy test for the skin
• Skin soreness

Besides these signs, if a person experiences mouth sores three times in a year then it is highly likely that they have this disease.

Once Behçet’s disease is diagnosed then the doctor is likely to recommend one or more of the following treatment procedures:

• Medication such as Corticosteroids to control the inflammation and pain.
• Medication to boost the immune system of the patient. This can reduce the time of flares and increase the time gap between two flares.
• Resting when symptoms appear or reappear.
• Physical activities. simple exercises like walking are the best for this kind of treatment.

There is no surgical method to treat this disease because doctors can’t change the blood vessels. Soreness can happen inside or outside of the skin. If the soreness is on the outside of the skin then cream or gel can be used to reduce the effects. Eye drops can also be used to control the inflammation of eyes.


This disease is one of the most challenging diseases in the world of medicine. Like many autoimmune conditions, its causes are still not fully understood and currently there is no cure. However, research into its causes and cures continues, so there is still hope for a positive outcome in the future.

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