Behcet’s disease or Turkish. Behcet’s disease is a relapsing progressive immune disorder that primarily affects Southeast Asian and Turkish men 30 years of age and older. The main symptoms are frequently recurring aphthae and disorders of the eyes, especially inflammation and pus accumulation. Several options are available for therapy, the most important being the administration of cortisone.
What is Behcet’s disease?
Behcet’s disease, a rheumatic disorder, affects the immune system and occurs in episodes. Symptoms that can be identified as this disease were described as early as ancient times. The first symptom was often recurrent aphthae on the oral or genital mucosa. In the further, then definite, course of symptoms, there are diseases of the eyes, especially accumulations of pus, which occur mainly in the anterior chamber of the eye, and inflammation of the iris. Rarely described are rheumatic symptoms such as skin redness and nodules, discomfort and inflammation of the joints, arterial vascular occlusion, and inflammation of the epididymis. Very rarely, brain inflammation occurs, 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 100000.
Causes
Although the disease has been known since ancient times, its causes and the reasons for its clustered occurrence in Turkey and Southeast Asia have not been elucidated to date. However, while there are concrete conjectures on the causes, no theories on the sites 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 immune system to such an extent that disturbed immune regulation occurs. This would condition the onset of disease because the body lacks the ability to clear inflammation and pus accumulation on its own.
Symptoms, complaints, and signs
Behcet’s disease is manifested by small, usually painful, skin patches in the mouth and intimate areas. These aphthae can occur singly or in larger groups and vary in appearance. They look like acne, vesicles or palpable nodules, hurt to the touch and are always inflamed. After some time, the skin areas scar and the skin peels off with scaling. In most cases, those affected also notice impaired wound healing, and there is secondary bleeding, oozing and infection in the case of injuries. The skin changes are often accompanied by conjunctivitis. In this process, the iris swells, resulting in tearing, itching and redness. Pus collections form in the anterior chamber of the eye, which eventually rupture and empty inward or outward. In severe cases, the patient goes blind as a result of the inflammation. The symptoms usually develop insidiously and increase in severity as Behcet’s disease progresses. 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. Sufferers feel increasingly unwell, and psychological complaints such as depression or inferiority complexes often develop as a result.
Diagnosis and course
Affected individuals with Behcet’s disease often consult a physician because they notice irritating wound healing behavior in themselves. The injured skin proves to be hyperactive, and there is severe redness and blistering around the wound. This symptom also serves the physicians consulted as the main criterion for the early detection of Behcet’s disease. The diagnosis is confirmed by means of the so-called “cat’s elbow test”. For this purpose, a physician injects 0.5ml of medical saline into the skin of the elbow and observes its reaction. If the patient has Behcet’s disease, the exuberant skin reacts to the injection with the formation of nodules and inflammatory reactions. If this test confirms the suspicion, blood analyses must be performed for further diagnosis in order to determine how much immunoglobulin is contained in the blood and how it is composed. Behcet’s disease is a chronic disease that progresses in episodes, initially manifested by aphthae and later by various eye diseases.If not intervened by a physician, the rheumatic disease can lead to blindness or severe inflammatory processes in the brain.
Complications
Behcet’s disease causes a variety of symptoms and complications. First and foremost, inflammation of the eyes can occur, causing pus to accumulate in the anterior chamber of the eye. In most cases, this accumulation leads to visual problems and, in the worst case, to complete blindness. The skin becomes itchy and reddened, which considerably reduces the quality of life of the affected person. Most people 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 may also be limited by Behcet’s disease, resulting in more frequent infections. Behcet’s disease can be treated relatively well with the help of medication. As a rule, no particular complications occur. In most cases, however, patients require prolonged treatment. Likewise, there is usually no reduction in the life expectancy of the affected person. Due to the weakened immune system, patients are more susceptible to various diseases.
When should you see a doctor?
The risk group for the disease includes, in particular, men over the age of 30 whose origin is Turkish or Southeast Asian. They should see a doctor as soon as they suffer from increased development of aphthae. Painful areas on the gums or mucous membranes in the mouth should be examined and treated. If the affected regions continue to spread or if there are further complaints, a visit to the doctor should be made. Caution is advised 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 outcome. Consultation with a doctor is necessary if pain develops, the wound enlarges or sterile wound care cannot be guaranteed. If the affected person suffers repeatedly from the various symptoms within a few weeks or months, a doctor should be consulted. If blisters form or other changes occur in the skin’s appearance, this is considered an indication by the organism of existing irregularities. Palpable lumps, scaly skin or pain on touch should be presented to a doctor. If wounds heal poorly or there is unusual postoperative bleeding, a doctor is needed. If there are emotional or mental abnormalities, a doctor’s visit is also necessary. Depressive moods or behavioral abnormalities should be evaluated by a physician as soon as they persist unabated for several weeks.
Treatment and therapy
Once the diagnosis of Behcet’s disease has been clearly established, standard therapy is initiated. In the acute stage, this consists of the administration of cortisone, either 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 episodes, the treating physician may decide to administer immunosuppressants additionally or alternatively. These reduce the functions of the immune system by restricting the growth of immune cells or blocking DNA growth. The dosage 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 are effective, therapies with infliximab or thalidomide are available as a last resort. Infliximab is considered a key drug for rheumatic diseases. Thalodomide became known by the names and negative experiences of thalidomide or Softenon, but in its current form and under strict safety controls, it promises good prospects for cure.
Prospect and prognosis
The prognosis in Behcet’s disease improves with increasing duration of disease. The course can be described as undulating, with the numerous aphthae in the mouth area or on the genitals in particular becoming less frequent with increasing age. Moreover, if only the skin is affected, sufferers do not have a lower life expectancy. The suffering pressure can rather be intensified by psychological problems, which arise due to the skin changes, which are perceived as disturbing.In rare cases, depression occurs, which otherwise worsens the prognosis. The mortality rate for Behcet’s disease is higher the earlier the disease first appears. Especially males in adolescent age as well as young adults have a much higher mortality rate than all other affected persons. Aneurysms in the pulmonary arteries in particular are associated with a high mortality rate of about one-fifth. Because they 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. However, with increasing age, the prognosis becomes more favorable even in this risk group because the disease becomes more inactive. Furthermore, the prognosis is poor if the eyes are involved, as approximately 25 to 50 percent of those affected go blind or become severely vision-impaired. Good medical care can prevent this.
Prevention
Because the causes of the disease have not been researched to date, no prevention methods are known. However, affected individuals should seek medical attention as soon as possible and on a regular basis to prevent severe disease progression.
Follow-up
The therapeutic goal in Behcet’s disease is a sustained and, if possible, complete suppression of the autoimmune inflammation of the blood vessels. In all cases, the activity of the alternating flaring and fading signs of disease diminishes over time. If the disease is more severe, 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 no complaints 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 bring Behcet’s disease under control because the time course and severity of relapses are incalculable. Therefore, optimal follow-up care is important in autoimmune vasculitis. The measures for aftercare following the acute therapy phase have the task of detecting secondary symptoms and recognizing a renewed attack in good time. Based on the clinical appearance, the medication dosages can be individually adjusted. Supportive measures in the follow-up of Behcet’s disease include a dietary regimen to stabilize the intestinal flora and strengthen the blood vessels. Regular check-ups, especially with dermatologists and ophthalmologists, allow early detection of skin changes or eye inflammations, but also give patients confidence in clarifying urgent questions. Follow-up treatment for Behcet’s disease is an important component in permanently consolidating the success of a therapy and adapting it to the individual course of the disease.
What you can do yourself
Because the disease progresses in relapses, it is difficult to issue a patent remedy for self-help. Many patients can live well and tolerably with the disease if the prescribed medication is taken regularly. For some patients, there are also times when they can manage without any medication at all. If another relapse occurs, the attending physician should be consulted in order to prescribe something to alleviate it, since severe pain is always associated with it. Anyone suffering from Behcet’s disease should in any case make sure to give their body enough rest. Sufficient sleep and a healthy, balanced diet will also contribute to a relatively stable course of the disease. In addition, sufferers should pay attention to 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 anonymity. In this way, everyone can exchange information with other sufferers about topics that perhaps should not be discussed in an official group. It is a help to hear the experiences of others in the particular situation. This makes it easier to deal with one’s own feelings and fears.
