The Behçet syndrome It is characterized by an inflammation of the blood vessels throughout the body, in addition to a series of signs that can be ignored; such as mouth and genital ulcers, eye inflammation, and skin rashes. However, the symptoms vary according to each patient and, in some cases, they can recover spontaneously..
For now, the exact causes of this disease are unknown, although there appears to be a genetic predisposition to it. For this reason, treatment is aimed at alleviating signs and symptoms and preventing serious complications..
Behçet's syndrome, which also appears in the medical literature as “BD” for its acronym in English “Behçet Disease”, arose in 1937 when a Turkish dermatologist named Hulusi Behçet described its symptoms for the first time. Although according to Rabinovich (2016), it is possible that it had already been described in the 5th century by Hippocrates.
This disease covers a series of aspects, that is why it is called multisystemic and is divided into neuro-behçet, ocular-Behçet and vasculo-Behçet according to the affected systems.
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The exact cause of this syndrome has not yet been detected. It seems that it has a genetic background, with some people being more predisposed to presenting it than others. Being predisposed to the syndrome means that, if a person carries the gene related to the disease, it can arise if they are exposed to certain triggering environments.
On the other hand, it seems that patients whose father or mother has this disease develop Behçet's syndrome at an earlier age, which is called genetic anticipation..
It has been shown that many of the patients with Behçet syndrome have more HLA (Human Leukocyte Antigens) in their blood than healthy people, especially the allele of the HLA-B51 gene.
It is not really known what role HLAs play in the disease, but knowing the role of these antigens can give us a clue; which consists of participating in the immune response, defending the body from potentially dangerous external agents.
In this way, it may be related to autoimmune disorders, which consist of a malfunction of the immune system. In this way, instead of protecting the body from possible threats, healthy cells are attacked and damaged..
Possible causes are currently being investigated, considering the role of other genetic markers, bacterial infections or viruses, and even thinking that it may be an auto-inflammatory disorder. That is, a condition in which the body is unable to regulate inflammatory processes.
In fact, it has been proven that infections can participate in the onset of the disease, since those affected by Behçet syndrome have a higher incidence of antibodies to hepatitis C virus, herpes simplex virus, and human parvovirus B19, in addition to streptococcal antigens. All this, together with environmental factors, could trigger the disease.
The symptoms of Behçet syndrome vary according to each person and affected areas of the body, and can disappear and reappear for no apparent reason. Generally, it seems that as time passes the symptoms become less severe.
The main symptoms of the disease include vasculitis, skin lesions, ulcers in the mouth and genitals, and eye problems that can lead to blindness. We describe the symptoms of Behçet syndrome in more detail below:
- Injuries (canker sores or painful sores) on the inner lining of the mouth and ulcers on the genitals. Canker sores heal on their own in about 3 weeks, although in this syndrome they tend to reappear. These are the most common signs of the disease.
- Skin problems which can be variable, with acne-like rashes appearing in some people and red nodules that protrude mainly on the legs in others. Folliculitis or inflammation of the hair follicles can also occur.
- Inflammation of the eyes or uveitis, inflaming the uvea, the vascular membrane that covers the eye. Uveitis can occur anterior (when the front of the eye or iris swells), posterior (the back of the eye), or panuveitis (when it affects the entire uvea).
This will cause the eyes to look red and each time they lose more visual acuity; in addition to pain, tearing and photophobia (intolerance to light). In people with the syndrome, this ocular involvement is just a symptom that can come and go.
- Joint problems, especially pain and swelling in the knees; although the wrists, elbows or ankles are also commonly involved. In this case, they are also symptoms that appear and disappear, lasting at most 3 weeks at a time. It can lead to arthritis in the long run.
- Involvement of blood vessels, More specifically, it is characterized by an inflammation of the arteries and veins that cause the arms or legs to become red, swollen and cause pain to the person. It can also cause blood clots and this leads to serious complications such as aneurysms, thrombosis, and narrowing or obstruction of the blood vessels..
Many times these vascular lesions can be accompanied by hypercoagulability, which occurs when the affected person's blood clots much faster than normal.
- Alterations in the central nervous system which can occur in up to 25% of children, being the most serious consequence of the syndrome. Specifically, brain inflammation occurs that causes an increase in intracranial pressure, leading to headaches, confusion, fever and loss of balance.
It can even cause meningoencephalitis, focal neurological disorders, neuropsychiatric symptoms such as hallucinations, or stroke..
Damage to the brain stem and cerebellum, cranial nerve palsy, or brain pseudotumors have been found in these patients..
- Pulmonary vasculitis, breathing difficulties, chest pains, cough, etc..
- Digestive problems such as abdominal pain, diarrhea, or bleeding in the stool.
- A study by Ilhan et al. (2016) it was shown that patients, who are active and suffer from this syndrome, are more fatigued than those affected who are inactive. In addition, they observed that fatigue was associated with depression and anxiety.
We can classify Behçet's disease into different types according to its most outstanding manifestations, which can vary for genetic and racial reasons. We distinguish between:
- Ocular (60-80% of patients). Where uveitis and other complications in vision predominate that would indicate the possible development of blindness.
- Neuro-Behçet (10-30% of patients) Involves the central nervous system progressively; leading to a poor prognosis. It encompasses meningitis or meningoencephalitis, psychiatric symptoms, neurological deficits, hemiparesis, and brain stem symptoms. Some of the patients go on to develop dementia.
- Vascular. Vascular complications occur in 7 to 40% of patients, and include arterial and venous thrombosis, occlusions of blood vessels, stenosis, and aneurysm..
Behçet syndrome is a rare disease, especially in the United States and Western Europe. Its prevalence occurs especially in Asia and the Middle East, specifically on the Silk Road.
In Turkey there is the highest prevalence rate of this disease, ranging between 80-370 cases per 100,000 individuals. This is followed by Japan, Korea, China, Iran and Saudi Arabia with a prevalence of 13-20 cases per 100,000 inhabitants; being the leading cause of blindness in Japan.
In northern Spain, a prevalence of 0.66 cases per 100,000 individuals has been estimated, while in Germany it stands at 2.26 cases per 100,000 inhabitants.
On the other hand, central nervous system involvement is more common in the United States and northern Europe..
It usually starts between 30 and 40 years of age, being very rare in young children. The mean age of onset of Behçet syndrome in Turkey is 11.7 years, while its neurological variant is 13 years.
Regarding sex, in the United States, Japan and Korea this syndrome is more common in women than in men (two women for each man), although the symptoms are usually less severe in them. In the countries of the Middle East, the opposite happens, there are more men affected and in a more serious way than women.
For the diagnosis of Behçet syndrome, one must be cautious, since some symptoms can occur in many other conditions and diseases that do not necessarily constitute this syndrome..
On the other hand, it is important to know that there are no tests specifically designed to detect Behçet's disease..
However, there are a series of diagnostic criteria based on the manifestations of this condition in order to detect it. Other medical tests will also be recommended to rule out other similar diseases or the level of involvement of the disease. These tests include:
- Blood test.
- Urine analysis.
- X-rays, Computed Tomography (CT), or Magnetic Resonance Imaging (MRI).
- Skin biopsy.
- Pathergy test (described below).
The diagnostic criteria used for Behçet syndrome are the following:
- A necessary sign for diagnosis is mouth ulcers, which have appeared at least 3 times during the period of one year..
In addition, it is essential to present at least 2 signs of the following:
- Genital ulcers that recur over time.
- Eye problems due to eye inflammation or uveitis.
- Acne-like skin rashes or sores.
- Give positive in a skin pathergy test, which consists of injecting the patient into the forearm a minimum amount of physiological saline, which should be safe for the person.
Then the reaction is observed one or two days later, being positive if a red bump forms on the skin, which would indicate that the immune system is not reacting properly. People with this disease do not heal well ulcers and wounds.
However, these criteria can be excessively strict, especially in some children who may have this syndrome and who normally will not develop ulcers in the mouth or genitals..
On the other hand, an attempt should be made to make a differential diagnosis with other diseases with which it can be confused, such as systemic lupus erythematosus, inflammatory bowel disease, Reiter's syndrome or herpes infections..
The prognosis for Behçet syndrome depends on your signs and symptoms. Its course is usually intermittent, with moments of remission and others in which symptoms appear. However, the disease has a natural tendency to disappear.
In patients with vision problems, improper treatment can lead to blindness 3 to 4 years after the onset of eye symptoms..
This disease usually leads to a significant decrease in the quality of life of those who suffer from it..
For now there is no cure for this syndrome, the treatment being focused on alleviating the symptoms of the syndrome that each person presents. For example, your doctor may prescribe medications that reduce the inflammation and discomfort caused by rashes, such as skin creams, gels, or ointments..
The most commonly used medications are those containing corticosteroids, which can be applied to skin lesions and ulcers, mouthwashes to relieve the pain of sores, eye drops, etc. This helps reduce discomfort when the disease is not so severe.
If topical medications do not work much, you can opt for colchicine, a drug that has been shown to be helpful in treating arthritis.
However, the signs and symptoms of Behçet syndrome tend to recur, and corticosteroids only eliminate the discomfort, but not what causes it. For this reason, doctors will usually also prescribe immunosuppressive drugs (which control the activity of the immune system, since it attacks healthy cells).
Some of these drugs are: azathioprine, cyclophosphamide or cyclosporine, also using interferon alfa-2b injected. All of them are useful for treating disorders in the central nervous system, while azathioprine has been shown to be very effective for treating major eye problems.
On the other hand, one must be cautious since these drugs by suppressing the action of the immune system can cause the person to contract infections more frequently. It can also lead to other side effects like high blood pressure or kidney or liver problems..
For those affected who are at a higher level of severity, drugs that block a substance called tumor necrosis factor (TNF) may be helpful for some symptoms of Behçet's disease. For example, etanercept or infliximab.
If vascular, neurological or gastrointestinal problems have been detected, it is usually recommended that the affected person undergo periodic analyzes in different specialists to control their situation and prevent it from worsening, in addition to knowing if the treatments are working or not. It is also important to keep this control in vision problems.
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