In Behcet's disease there is an inflammation of the small blood vessels, a so-called vasculitis. The disease is named after the Turkish doctor Hulus Behcet, who first described the disease in 1937.
In addition to the vascular inflammation, the disease may also be expressed in other organ systems. The cause is not clear to this day.
Behcet's disease is a systemic disease, which means that the disease can spread to the whole organ system. As a result, each patient may experience different symptoms in different degrees. These include:
The main symptoms of Behcet's disease include the painful aphthae in the mouth, which usually occur in the back of the mouth, as well as genital ulcers and ulcers, as well as the inflammations of the middle eye skin.
Other concomitant symptoms are various skin lesions in some individuals. It may be acne-like pustules, hair follicles, ulcers, knots or even affect skin irritation. Because Behcet's disease progresses in spurts, there are always symptom-free intervals. Early signs or symptoms are especially the recurring aphthae.
One of the main symptoms of Behcet's disease is the aphthae. It is a painful damage to the skin or the mucous membrane . It forms an inflammatory rim around the small ulcer. Their diameter is about 1 mm to 30 mm. They occur very quickly and can cause severe pain.
But they are not contagious.
In Behcet's disease, aphthae occur at a very early stage of the disease. Often they are the first hallmark of systemic disease. The aphthae are usually found in the genital area or in the mouth.
The aphids heal after some time, but return again. Scars can remain in very large apices. There is no drug treatment against the formation of aphthae only for pain relief, various agents can be used.
One of the first symptoms of Behcet's disease is genital ulcers.
An aphthous ulcer is a painful injury to the mucous membrane or the skin in the genital area, surrounded by an inflammatory rim. These are therefore small ulcers. In boys, these occur mainly on the scrotum. In adulthood, these ulcers usually leave scars.
In the female sex, the outer genitals are usually affected. Again, it usually comes to scarred healing.
Symptoms of Behcet's disease are common in the eye.
These symptoms are often the main problem of the disease. It can come to pus accumulation in the anterior chamber, a so-called hypopyon. In addition, an inflammation of the iris, a uveitis come. This inflammation can, if it persists for a very long time, lead to a loss of sight. A dreaded complication represents a blindness of those affected.
To exclude complications should therefore be a regular ophthalmological examination of those affected.
Behcet's disease can also cause symptoms on the intestines or on the digestive tract. Much like the ulcers on the oral mucosa can be found here also defects in the mucous membrane of the intestine. Symptoms include malaise, severe abdominal cramps, black stools, or bloody diarrhea.
It is possible that the symptoms resemble the clinical picture of Crohn's disease (inflammatory bowel disease).
Patients suffering from Behcet's disease may also experience joint discomfort. These are triggered by inflammation in the joint. This inflammation is usually very mild and usually heal without leaving permanent damage after a few weeks.
Most affected are the large body joints. Typically, the knee, ankle, wrist or elbow. The symptoms at the joints can be expressed by swelling, pain, stiffness and restricted movement.
Further information: Rheumatoid arthritis
The therapy of Behcet's disease consists mainly in the administration of cortisone. It is administered either in tablet form or intravenously, depending on, among other things, the condition and severity of the symptoms of those affected.
Cortisone inhibits inflammation in the body.
Also, a local application of cortisone, for example in the form of an ointment in externally visible inflammation such as the eye is possible.
In the case of very severe disease or relapses, an immunosuppressant may additionally or alternatively be prescribed. The immunosuppressant reduces immune system function by limiting the growth of immune cells. A combination of both drugs is often given in case of pathological involvement of the central nervous system.
Behcet's disease is one of the chronic diseases. Often, the disease occurs in spurts, that is, those affected have phases in which the symptoms are only slightly to barely perceptible and then also phases in which there is a strong expression of typical for the disease complaints. So there is an opposite to acute diseases no definite endpoint. In the case of Behcet's disease, basically only the symptoms and not the causes can be treated.
An exact duration of the drug treatment can not be fixed flat rate. However, these are years or the treatment can also last the entire life of a person affected. The disease weakens the immune system, which can make the patient more susceptible to various other conditions.
If the disease has been diagnosed as a child, it may be that the symptom-free sections are getting longer and longer. It is also observed in some patients, a decay of Behcet's disease. However, it is not possible to provide a generalized prognosis, the disease is too individual.
It is also possible that complications occur in the course of the disease, especially the eyes are affected. However, a reduced life expectancy due to the disease can not be expected.
Unfortunately, it has not yet been possible to clarify the causes of Behcet's disease.
Presumably, however, the cause is an autoimmune disease, which causes inflammation of the vessels. For this reason, the disease is counted among rheumatic forms, as even here autoimmune reactions lead to inflammation, for example in the joints.
It is also a genetic predisposition suspected in those affected, as the disease occurs frequently in certain local areas. Due to genetic predisposition, the immune system, which is supposed to protect the body from invading viruses and bacteria, is no longer functioning properly. It recognizes the body's own tissue as an intruder and therefore attacks it. So it fights itself. This leads to the inflammatory reactions.
In Behcet's Disease, these disturbed autoimmune reactions are found in the small vessels. As a result, the inflammation occurs especially in the skin, in the mucous membrane (slime-forming tissue, which lines the digestive, genital and urinary organs) and in the eye.
Scientists suspect that external influences such as bacterial and viral inflammation also play a role.
The diagnosis of patients suffering from Behcet's disease is usually made after the appearance of externally visible symptoms. These include in particular the aphthae in the mouth as well as the genital aphthae and other typical skin lesions.
In addition, a test can clarify whether you suffer from Behcet's disease or not. This test is called pathergie test. Saline is injected directly under the skin. A short time later, when there is a skin reaction with nodules and inflammation, patients are most likely suffering from Behcet's disease.
In addition, immunological blood tests or an MRI can be made for diagnosis.
The HLA system is a group of proteins that are found on the surface of cells and that are important for the function of the immune system. The abbreviation HLA comes from the English (Human Leukocyte Antigen).
Patients with Behcet's disease may undergo HLA typing. It is determined if the patient HLA B51 is positive. This is the case in 70% of all Behcet Disease patients.
Behcet's disease is currently not contagious. This is because an autoimmune disease, a genetic defect, is described as the probable cause. The immune system, which is actually there to fight viruses, bacteria or fungi, recognizes the body's own tissue as "foreign" and begins to fight it. This malfunctioning of the immune system is anchored in the genes and therefore not contagious.
Also, the symptoms of this disease are not contagious. These include the genital or mouth aphthae.
Contrary to popular belief, there is no risk of infection in the aphthae.