Behcet Syndrome, or Silk Road Disease

Diagnosis of many diseases from a number of systemic vasculitis is significantly hampered because their symptoms are very similar to the symptoms of other disorders.

One of them is called Behcet's syndrome, and its main feature is the involvement of not only vessels and arteries, but also other organs in the inflammatory process.

What diagnostic methods are used to determine this disease, and what kind of help should the patient provide?


  • 1 Description
  • 2 Causes and Risk Factors
  • 3 Stages of
  • 4 Dangers and Complications
  • 5 Symptoms of
  • 6 When to see a doctor?
  • 7 Diagnosis
  • 8 Treatment
  • 9 Forecasts and prevention


Behcet's syndrome refers to the chronic recurrent disease that mainly affects the arteries, small and medium veins, skin, mucous membranes and some internal organs.

For the first time it was described in 1937 by the Turkish dermatologist Hulusi Behcet, but mention of a violation with similar symptoms is in the earlier works of the Greek therapist Adamantiada.

According to statistics, most often this disease occurs among Asians and Europeans , who live in the zone of the 30th 45th latitude. Geographically, this area corresponds to the legendary Silk Road, so Behcet's syndrome is sometimes called a similar name( illness of the Silk Road).

Causes and Risk Factors

The exact etiology of the disease is not yet clear, but even Behcet himself spoke of his viral origin. Subsequently, a specific virus was isolated from the patient's blood, but there is no direct evidence that he is the cause of the syndrome.

There are theories that causative agents of herpes and tuberculosis can precede and burden the course of the disease.

The autoimmune mechanism of its development has been discussed in the last decade: often patients are diagnosed with antigens HLADRW52 and B51 , and the risk of occurrence of Behcet's syndrome is especially great in the presence of two of these antigens simultaneously. In addition, the reported cases of familial morbidity and regional character indicate the role of genetic factors in the development of the disease.

The prevalence of Behçet's disease in men and women is not uniform : in the Middle East it affects mainly men, but in Japan and Korea, women predominate among the patients. The age of most patients does not exceed 30-40 years.

Stages of

The main difficulty in diagnosing a disease is that its clinical course can be very diverse .Between different stages, that is, the appearance of the first symptoms and the addition of others, a considerable time can pass, and by this time the first signs can completely disappear.

Patients with this diagnosis often experience spontaneous relapses and periods of remission without any pathological symptoms.

Dangers and complications of

The probability of complications in Behcet's disease largely depends on its severity, the patient's condition and other factors. In some cases, it passes for a person almost imperceptibly, while in others it leads to serious complications and even death. The causes of death are usually:

  • vascular lesions of the CNS;
  • meningoencephalitis;
  • thromboses of different localization;
  • perforation of gastrointestinal ulcers;
  • aneurysm ruptures;
  • embolism.

In addition, the common complication of the disease are eye lesions( uveitis), which can cause a loss of vision .

Symptoms of

Symptoms of Behcet's syndrome are very variable, which makes them divided into main and secondary, and they can arise not simultaneously, but with a difference of several weeks, months or even years. The main signs of the disease include:

  • Aft( ulcerative) stomatitis .Practically in 100% of patients the disease begins with the appearance of shallow painful ulcers on the oral mucosa. They have a small size( from 2 to 10 mm) and are localized on the inside of the cheeks, gums, lips and tongue, less often - in the throat and nose.

    These ulcers look like this: a necrotic yellowish base, surrounded by a red rim, that is, practically no different from usual aft. Usually, such lesions are observed for some time( several days or weeks), and then heal without a trace or leave behind small scars.

  • Lesions of the genitals. Genital ulcers( single or multiple) with Behcet's disease resemble ulcers that form in the mouth, and occur in 80% of cases. In women, they are located on the labia and vaginal mucosa, and in men - mainly on the scrotum and less often on the skin of the penis.

    Lesions can be absolutely painless, and are often found only during preventive examinations or in connection with other complaints. For example, if the bladder is involved in the process, the patient may experience symptoms of cystitis.

  • Eye lesions .One of the most serious and dangerous symptoms of Behcet's syndrome is a damage to the organs of vision, which is usually manifested as acute uveitis( inflammation of the eye vessels) with the formation of purulent exudate( approximately 2/3 of patients).It is accompanied by lacrimation, photophobia, changes in the fundus and generalized lesions of the retina.

    The course of uveitis is usually characterized by five major stages: systemic manifestations that do not affect the eye tissues, iridocyclitis, severe inflammation, severe vision impairment and cicatricial changes leading to complete blindness.

Eye lesions, genital ulcers and stomatitis are the classic "triad" of Behcet's syndrome, which is the determining factor in the diagnosis.

In addition, there are minor symptoms of the disease, which include:

  • Skin lesions .Eruptions in the form of pustules, erythematous papules and vesicles are characteristic for 70% of patients. Outwardly they resemble a knotty erythrema, but often have some peculiarities. Eruptions can be localized on the hands and in some cases have signs of ulceration, necrosis and suppuration of the skin.
  • Joint lesions .In 50% of the patients there are signs of arthritis in the large joints( knee, wrist, elbow), which are not accompanied by tissue destruction.
  • Manifestations of vasculitis .Inflammation of the walls of the vessels manifests hemorrhages on the skin and mucous membranes, bleeding from the gastrointestinal tract, impurities of blood in the sputum.

Other symptoms include headaches, frequent fevers, relapsing tonsillitis, epididymitis, swelling of the salivary glands, weight loss, neurological disorders, meningeal conditions.

When should I see a doctor?

Since the signs of Behcet's syndrome are very diverse and can be stretched in time, patients very rarely associate them with one disease.

In any case, if you have any anxiety symptoms( especially if they are associated with lesions of the mucous membranes, skin and eyes), the patient should be consulted by several specialists: therapist, infectious disease specialist, venereologist, ophthalmologist and rheumatologist ,organism.


The main diagnostic criterion in diagnosing is the presence of recurrent aphthous stomatitis in combination with two( at least) symptoms from the main .

Laboratory diagnostic methods mainly include blood tests of , which reveal an increase in ESR, fibrin level, gamma globulin and alpha-2, seromucoid, the emergence of PSA.In the study of synovial fluid, neutrophils predominate, indicating an inflammatory process.

In case of suspected Behcet's syndrome, the patient may be assigned skin test to the patient: the skin is pierced with a sterile needle to a depth of about 5 mm, after which the puncture site is observed. If there is a disease within two days, an erythrematous papule of about 2 mm in size is formed there.

Patients undergo joint radiography, which, with Behcet's disease, does not show any destructive changes-this factor plays a very important role in differential diagnosis.

Finally, in the process of study of the fundus of patients, it is possible to notice changes characteristic of this form of systemic vasculitis: widening of the retinal vasculature, fuzzy contours of the optic nerve, swelling of the retinal focuses, and at later stages - foci of acute exudation, retinal atrophy, obliterationvessels.

Differential diagnosis is performed with systemic lupus erythematosus and other forms of systemic vasculitis .So, with Reiter's syndrome, the onset of the disease is characterized by diarrhea, lesions of the genitourinary system and the mucous membrane of the mouth, which are accompanied by severe pain in the joints, which is not typical for Behcet's disease.

Treatment of

There is no specific treatment for Behcet's syndrome today, therefore therapeutic measures are aimed at relief of symptoms, relief of the condition of the patient and prevention of complications.

  1. If the disease manifests primarily skin lesions , patients are prescribed levamisole or immunosuppressors, non-steroidal anti-inflammatory drugs, ointments with glucocorticosteroids externally.
  2. In the systemic course of the , when the general condition is disturbed in patients, eye injuries, fevers and neurological disorders are observed, doctors use prednisolone, cytostatics, azathioprine, cyclophosphamide.
  3. In thrombophlebitis , reception of anticoagulants, as well as acetylsalicylic acid in high doses is indicated.
  4. Because there are no facts that disprove the viral etiology of the disease, patients can be prescribed appropriate therapy: anti-herpes drugs, antibiotics, vitamins .

Learn more about Behcet's syndrome from video:

Forecasts and prevention of

Forecasts for Behcet's disease depend on the organs and systems that are involved in the pathological process. With the isolated form of disease, when only the skin and mucous membranes are affected, the prognosis is generally favorable.

In the case of development of uveitis , the patient's quality of life can be worsened by partial or total blindness, and the most dangerous situations are the lesions of the central nervous system and vessels - in such cases, approximately 5% of patients die from developed complications. Due to the unclear etiology of the disease, its prevention measures have not yet been developed.

Behcet's syndrome is a rare, but rather complex disease, and the quality of life of patients largely depends on their attentive attitude to their own health.

Therefore, if you have any anxiety signs, you should not only immediately go to the doctor and undergo appropriate studies, but must also monitor your condition to prevent the admission of new symptoms of and the development of complications.