Behcet's disease

Behcet's disease or turk. Behçet's disease is a relapsing immune disease that mainly affects Southeast Asian and Turkish men aged 30 and over. The most important symptoms are frequently recurring aphthae and diseases of the eyes, especially inflammation and accumulations of pus. Various options are available for therapy, the most important of which is the administration of cortisone.

What is Behcet's Disease?

The rheumatic disease Behcet's disease affects the immune system and occurs in spurts. Symptoms that can be recognized as this disease were already described in ancient times. The first symptom was often recurring canker sores on the oral or genital mucosa.

In the further course of the symptoms, which is then unambiguous, there are diseases of the eyes, in particular accumulations of pus, which occur primarily in the anterior chamber, and inflammation of the iris.

Rheumatic symptoms such as reddening of the skin and lumps, discomfort and inflammation of the joints, arterial vascular occlusions and inflammation of the epididymis are rarely described. Brain inflammation occurs very rarely, which can lead to coordination disorders, headaches, spasticity and impaired consciousness.

Behcet's disease can also occur in adolescents and is then monosymptomatic. The incidence of the disease is less than 1 in 100,000.

causes

Although the disease has been known since ancient times, the reasons for it 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 about the places of occurrence could be found.

Scientists suspect that the interplay 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 impaired. This would cause the outbreak of the disease 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, mostly painful areas of skin in the mouth and genital area. These canker sores can appear individually or in larger groups and have different characteristics. They look like acne, vesicles or palpable nodules, they hurt to the touch and are always inflamed. After a while, the areas of the skin become scarred and the skin flakes off.

In most cases, those affected also notice impaired wound healing, and injuries lead to secondary bleeding, oozing and infections. The skin changes are often accompanied by conjunctivitis. The iris swells, resulting in tears, itching and redness. In the anterior chamber of the eye, collections of pus form, which eventually break up and empty inwards or outwards.

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. The sick feel increasingly unwell, and as a result emotional complaints such as depression or inferiority complexes often develop.

Diagnosis & course

Affected by Behcet's disease often turn to a doctor because they notice irritating wound healing behavior. The injured skin turns out to be hyperactive, there is severe reddening of the skin and blistering in the area around the wound. This symptom also serves as the main criterion for the early diagnosis of Behcet's disease by the doctors consulted.

The diagnosis is confirmed using the so-called "cat's elbow test". For this, a doctor injects 0.5 ml of medical saline solution into the skin of the elbow and observes its reaction. If the patient suffers from Behcet's disease, the excess skin reacts to the injection with nodules and inflammation.

If this test confirms the suspicion, blood analyzes must be carried out for further diagnosis to determine how much immunoglobulin is in the blood and how it is made up.

Behcet's disease is a relapsing chronic disease that initially manifests itself in canker sores 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.

Complications

Behcet's disease causes various complaints and complications. Primarily, it can cause inflammation of the eyes, causing pus to collect in the anterior chamber. This accumulation leads to visual problems in most cases and in the worst case to complete blindness. Itching and redness develop on the skin, which significantly limit the quality of life of the person affected.

Most of them feel uncomfortable and are often ashamed of these complaints. Behcet's disease can also lead to decreased 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 the help of medication. As a rule, there are no particular complications. In most cases, however, the patients are dependent on lengthy treatment. Likewise, there is usually no reduced life expectancy for the person affected. Due to the weakened immune system, patients are more susceptible to various diseases.

When should you go to the doctor?

The risk group for the disease particularly includes men aged 30 and over, whose origin is Turkish or Southeast Asian. You should consult a doctor as soon as you suffer from aphthous ulcers. Sore spots on the gums or mucous membranes in the mouth should be examined and treated. If the affected regions spread further or if there are further complaints, a doctor should be consulted. Caution should be exercised if there is inflammation, itching, open wounds or the formation of pus. In severe cases there is a risk of blood poisoning with a potentially fatal outcome. Consultation with a doctor is necessary if pain develops, the wound enlarges or sterile wound care cannot be guaranteed.

If the person concerned repeatedly suffers from the various symptoms within a few weeks or months, a doctor should be consulted. If vesicles form or if there are other changes in the complexion, this is an indication of the organism for existing irregularities. Palpable lumps, flaky skin, or pain to the touch should be presented to a doctor. If wounds heal poorly or there is unusual bleeding, a doctor is needed. A visit to the doctor is also necessary in the event of emotional or psychological problems. Depressive moods or behavioral problems should be assessed by a doctor as soon as they persist for several weeks.

Treatment & Therapy

Is the diagnosis Behcet's disease clearly proven, standard therapy is initiated. In the acute stage, this consists of the administration of cortisone, intravenously or in tablet form, depending on the severity and individual needs of the patient. 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 attacks, the attending physician may decide to additionally or alternatively administer immunosuppressants. These reduce the functions of the immune system by restricting the growth of immune cells or blocking DNA growth. The dose level and frequency of intake depend on the severity of the disease.

If neither the cortisone therapy nor the immunosuppressants or the combination of both preparations work, then the last options available are therapies with infliximab or thalidomide. Infliximab is a key drug in rheumatic diseases. Thalodomid was known under the names and negative experiences with Contergan or Softenon, but in its current form and under strict security controls it promises good prospects of a cure.

Outlook & forecast

The prognosis for Behcet's disease improves as the duration of the disease increases. The course can be described as wavy, with the numerous aphthae in the mouth or on the genitals becoming rarer with increasing age. If only the complexion is affected, those affected have no shorter life expectancy. The psychological problems that arise as a result of the skin changes that are perceived as annoying can increase the level of suffering. In rare cases, depression develops, which otherwise worsens the prognosis.

The earlier the disease first occurs, the higher the death rate from Behcet's disease. Young men and young adults in particular have a much higher death rate than anyone else affected. Aneurysms in the pulmonary arteries are associated with a high mortality rate of around one fifth. Since these are rarely diagnosed before they take effect, the prognosis is correspondingly poor. Neuronal involvement or ulceration in the digestive tract or other organs also contribute to the increased mortality rate.

With increasing age, however, the prognosis for this risk group becomes more and more favorable because the disease becomes more inactive. Furthermore, the prognosis is poor if the eyes are involved, because around 25 to 50 percent of those affected go blind or their eyesight is severely restricted. Good medical care can prevent this.

prevention

Since the causes of the disease have not yet been researched, no prevention methods are known. However, those affected should seek medical help as soon as possible and regularly in order to prevent serious disease progression.

Aftercare

The therapeutic goal for 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 alternately flickering and subsiding symptoms of the disease decreases over time. If the symptoms are more pronounced with inflammation of the eyes, 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 complaints for at least two years and are without symptoms, preparations to reduce inflammatory processes or immunosuppressive therapies are discontinued in individual cases. However, it is often difficult to bring Behcet's disease under control because the timing and severity of the relapses are incalculable.

Optimal follow-up care is therefore important for autoimmune vasculitis. The follow-up treatment measures following the acute therapy phase have the task of identifying sequelae and recognizing a new flare in good time. The drug dosages can be individually adjusted based on the clinical appearance. A nutritional style to stabilize the intestinal flora and strengthen the blood vessels supports the follow-up care of Behcet's disease.

Regular check-ups, especially by the dermatologist and ophthalmologist, enable the early detection of skin changes or eye inflammation, but also give those affected the security of clarifying urgent questions. Follow-up treatment for Behcet's disease is an important component 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 bouts, it is difficult to come up with a panacea for self-help. Many patients can live well and tolerably with the disease if the prescribed medication is taken regularly. For some sick people there are also times when they can cope without any medication at all. If another episode occurs, the attending physician should be consulted to prescribe something for relief, as severe pain is always associated with it.

Anyone who suffers from Behcet's disease should make sure that they give their body enough rest. Sufficient sleep and a healthy, balanced diet will also contribute to a relatively stable course of the disease. Sick people should also lead a regular lifestyle in order to save their body unnecessary stress.

Furthermore, sick people are advised to join a self-help group or therapy group. There are also some internet forums that offer a certain anonymity. In this way everyone can exchange ideas with other affected parties about topics that may not be discussed in an official group. It helps to hear the experiences of others in the respective situation. This is a better way to deal with one's own feelings and fears.