Behcet’s disease or Turkish Behçet’s disease is a relapsing immune disease that primarily affects Southeast Asian and Turkish men over the age of 30. The most important symptoms are frequently recurring aphthous ulcers and diseases of the eyes, especially inflammation and accumulations of pus. Various options are available for therapy, the most important being the administration of cortisone.
What is Behcet’s disease?
The rheumatic disease Behcet’s disease affects the immune system and occurs in episodes. Symptoms that can be recognized as this disease were already described in ancient times. The first symptom was often recurring aphthae on the oral or genital mucosa. See lawfaqs for Definitions of Impingement Syndrome.
In the further, then clear, course of symptoms, there are diseases of the eyes, in particular accumulations of pus, which occur primarily in the anterior chamber of the eye, and inflammation of the iris.
Rheumatic symptoms such as skin redness and knots, discomfort and inflammation of the joints, arterial vascular occlusion and inflammation of the epididymis are rarely described. Inflammation of the brain, which can lead to coordination disorders, headaches, spasticity and disturbances of consciousness, occurs very rarely.
Behcet’s disease can also occur in adolescents and is then monosymptomatic. The incidence of the disease is less than 1 in 100,000.
Although the disease has been known since antiquity, its causes and the reasons for its frequent occurrence in Turkey and Southeast Asia have not yet been clarified. However, while there are concrete guesses as to the causes, no theories have been found as to where it occurred.
Scientists suspect that the interaction of genetic predisposition and autoimmune diseases with bacterial or viral inflammation weakens the body and the immune system to such an extent that immune regulation is disturbed. This would cause the disease to break out because the body lacks the ability to break down inflammation and accumulations of pus on its own.
Symptoms, Ailments & Signs
Behcet’s disease manifests itself as small, usually painful patches of skin in the mouth and genital area. These canker sores can occur individually or in larger groups and have different characteristics. They look like acne, blisters, or palpable nodules, are painful to the touch, and are always inflamed. After some time, the skin scars and the skin peels off in a scaly manner.
In most cases, those affected also notice impaired wound healing, injuries lead to postoperative bleeding, weeping and infections. The skin changes are often accompanied by conjunctivitis. The iris swells, resulting in tears, itching and redness. Collections of pus form in the anterior chamber of the eye, which eventually ruptures and empties inward or outward.
In severe cases, the patient becomes blind as a result of the inflammation. The symptoms usually develop insidiously and increase in the course of Behcet’s disease. With appropriate treatment, the health problems subside within a few days or weeks. If the rheumatic disease is not treated, it can lead to scarring, chronic pain and disorders of the immune system. Patients feel increasingly unwell, and this often results in psychological problems such as depression or an inferiority complex.
Diagnosis & History
Sufferers of Behcet’s disease often turn to a doctor because they notice an irritating wound healing behavior. The injured skin turns out to be hyperactive, there is severe reddening of the skin and blistering in the vicinity of the wound. This symptom also serves as the main criterion for the early detection of Behcet’s disease by the doctors consulted.
The diagnosis is secured using the so-called “cat’s elbow test”. For this purpose, a doctor injects 0.5 ml of medical saline into the skin of the elbow and observes its reaction. If the patient suffers from Behcet’s disease, the protruding skin reacts to the injection with the formation of nodules and an inflammatory reaction.
If this test confirms the suspicion, blood analyzes must be carried out for further diagnosis in order to determine how much immunoglobulin is in the blood and what its composition is.
Behcet’s disease is a chronic disease that develops in relapses, which first manifests itself in the form of aphthae and later in various eye diseases. If there is no medical intervention, the rheumatic disease can lead to blindness or severe inflammation in the brain.
Behcet’s disease causes various symptoms and complications. First and foremost, it can lead to inflammation of the eyes, so that pus collects in the anterior chamber of the eye. In most cases, this accumulation leads to visual problems and in the worst case to complete blindness. Itching and redness develop on the skin, which significantly reduces the quality of life of those affected.
Most of them feel uncomfortable and are often ashamed of these symptoms. Behcet’s disease can also lead to reduced self-esteem or inferiority complexes. It is not uncommon for patients to suffer from psychological limitations or depression as a result. Wound healing can also be restricted by Behcet’s disease, so that infections occur more frequently.
Behcet’s disease can be treated relatively well with medication. Special complications usually do not arise. In most cases, however, patients are dependent on lengthy treatment. Likewise, there is usually no reduced life expectancy of those affected. The weakened immune system makes patients more susceptible to various diseases.
When should you go to the doctor?
The risk group for the disease includes men over the age of 30 whose origin is Turkish or Southeast Asian. You should consult a doctor as soon as you suffer more from the development of aphthous ulcers. Sore spots on the gums or the mucous membranes in the mouth should be examined and treated. If the affected regions spread further or if further symptoms occur, a doctor should be consulted. Caution should be exercised in the event of inflammation, itching, open wounds or the formation of pus. In severe cases, there is a risk of blood poisoning with a potentially fatal course. Consultation with a doctor is necessary if pain develops, the wound enlarges or sterile wound care cannot be guaranteed.
If the person affected repeatedly suffers from the various symptoms within a few weeks or months, a doctor should be consulted. If blisters form or if there are other changes in the complexion of the skin, this is considered an indication of the organism for existing irregularities. Any palpable lumps, scaly skin, or tenderness to the touch should be presented to a doctor. If wounds heal poorly or if there is unusual post-bleeding, a doctor is needed. In the case of emotional or mental abnormalities, a doctor’s visit is also necessary. Depressive moods or behavioral problems should be evaluated by a doctor as soon as they persist unabated for several weeks.
Treatment & Therapy
If the diagnosis of Behcet’s disease is clearly established, standard therapy is initiated. In the acute stage, this consists of the administration of cortisone, depending on the severity and individual needs of the patient, either intravenously or in tablet form. Cortisone inhibits inflammation in the body and the growth of affected cells and can thus interrupt or weaken the disease cycle.
In very severe cases and frequently recurring flare-ups, the doctor treating you may decide to administer immunosuppressants in addition or as an alternative. These reduce the functions of the immune system by restricting the growth of immune cells or blocking DNA growth. The dose and frequency of administration depend on the severity of the disease.
If neither the cortisone therapy nor the immunosuppressants or the combination of both preparations work, the last options are therapies with infliximab or thalidomide. Infliximab is considered the central drug in rheumatic diseases. Thalodomid became known under the names and negative experiences with Contergan or Softenon, but in its current form and under strict safety controls it promises good prospects of a cure.
Outlook & Forecast
The prognosis for Behcet’s disease improves as the disease progresses. The course can be described as wavy, with the numerous aphthae in the mouth area or on the genitals becoming rarer with increasing age. If only the skin is affected, those affected do not have a reduced life expectancy. The level of suffering can be increased by psychological problems that arise due to the skin changes that are perceived as disturbing. Rarely, depression occurs, which otherwise worsens the prognosis.
The earlier the disease first appears, the higher the mortality rate for Behcet’s disease. Especially young men and young adults have a much higher mortality rate than all other affected people. Aneurysms in the pulmonary arteries are associated with a high mortality rate of about one fifth. Since these are rarely diagnosed before they take effect, the prognosis is correspondingly poor. Neuronal involvement or ulceration of the digestive tract or other organs also contribute to the increased mortality rate.
With increasing age, however, the prognosis also becomes more favorable for this risk group because the disease becomes less active. Furthermore, the prognosis is poor if the eyes are affected, because around 25 to 50 percent of those affected go blind or their vision is severely restricted. Good medical care can prevent this.
Since the causes of the disease have not been researched to date, no prevention methods are known. However, those affected should seek medical help as quickly and regularly as possible in order to prevent serious progression of the disease.
The goal of therapy in Behcet’s disease is a lasting and as complete as possible suppression of the autoimmune inflammation of the blood vessels. As a rule, the activity of the alternating flare-ups and fading symptoms decreases over time. In more severe cases, with eye inflammation, thrombosis and involvement of the nervous system or gastrointestinal tract, long-term follow-up treatment with anti-inflammatory drugs such as infusions and tablets is required.
If patients have had no symptoms for at least two years and are asymptomatic, preparations to reduce inflammatory processes or immunosuppressive therapies are discontinued in individual cases. However, it is often difficult to get Behcet’s disease under control because the timing and severity of the flare-ups are unpredictable.
Optimal follow-up care is therefore important in autoimmune vasculitis. The post-treatment measures after the acute phase of therapy have the task of identifying sequelae and recognizing a new attack in good time. Based on the clinical appearance, the drug dosages can be individually adjusted. A diet that stabilizes the intestinal flora and strengthens the blood vessels is helpful in the aftercare of Behcet’s disease.
Regular check-ups, especially by dermatologists and ophthalmologists, enable early detection of skin changes or eye infections, but also give those affected security when urgent questions are clarified. The follow-up treatment for Behcet’s disease is an important building block in order to permanently consolidate the success of a therapy and to adapt it to the individual course of the disease.
You can do that yourself
Since the disease progresses in phases, it is difficult to issue a panacea for self-help. Many patients can live comfortably and tolerably with the disease if the prescribed medication is taken regularly. There are times when some sufferers can get by without medication at all. If a relapse occurs, the doctor treating you should definitely be consulted in order to prescribe something for relief, as severe pain is always associated with it.
If you suffer from Behcet’s disease, you should definitely make sure that you give your body enough rest. Adequate sleep and a healthy, balanced diet will also contribute to a relatively stable course of the disease. In addition, sufferers should ensure a regular lifestyle in order to spare their bodies unnecessary stress.
Furthermore, patients are advised to join a self-help group or therapy group. There are also some Internet forums that offer a certain degree of anonymity. In this way, everyone can exchange ideas with other affected people about topics that perhaps should not be discussed in an official group. It is a help to hear the experiences of others in the respective situation. This way you can better deal with your own feelings and fears.