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Behcet's disease (Adamantiades-Behçet) is a rare systemic vascular disease that affects only a dozen people in our country. It begins with innocent mouth ulcers and may lead to the involvement of the central nervous system. What are the other symptoms of Behcet's disease? Can it be treated?

Behcet's diseasetosystemic vasculitis , with characteristic recurrent changes in the skin and mucosa. The disease is named after the Turkish dermatologist Hulusi Behçet, who diagnosed the symptoms of the disease in one of his patients. in 1936 he described its symptoms as a new disease entity in the Archives of Skin and Veneral Diseases. In 1947, the name was officially adopted at the International Dermatological Congress in Geneva.

The incidence of Behcet's disease varies by region. The highest incidence is observed in countries along the so-called the silk route, i.e. the trade route known since antiquity, through which silk and other products from China and Japan were transported to Europe. The first symptoms typically appear in the 3-4th decade of life. The majority of patients from Japan, Korea and Germany are women, while in the countries of the Middle East, men are dominant. It is a rare disease in Poland, so far only a dozen or so cases have been described, and the incidence is similar in both sexes. The actual number of patients may be greater, because due to diagnostic difficulties, only typical and full-blown forms of the disease are diagnosed.

Behcet's disease: causes

The causes of the disease are still unclear. There are many arguments for the genetic background of the disease. It occurs in certain areas and in certain populations. Due to the significant severity of the disease in the Turkish silk trade community, it was theorized that the HLA-B5101 and HLA-B5108 histocompatibility antigens predispose to the syndrome.

Patients also have changes in the immune system: increased production of pro-inflammatory cytokines, circulating immune complexes, antibodies against mucosa cells or antibodies against endothelial cells. However, it is not clear whether they are a cause or a secondary symptom of the disease.

Roleinfectious agents in initiating Behcet's disease have not been confirmed, but there are voices thatStreptococcus sanguis and oralis ,Saccharomyces cerevisiaeand herpes virus type I may call.

Behcet's disease: symptoms

  • Oral aphthas (97-99%)

Usually, the appearance of painful mouth ulcers is the first symptom of the disease. The recurrent nature of lesions is characteristic - more than 3 a year - which heal spontaneously for up to 3 weeks without leaving any scars

  • Genital ulcers (85%)

In men, they are found on the scrotum, in women on the labia, but also in the vagina and on the cervix. They are deep, very painful and difficult to treat. They often recur and, unlike aphthas in the mouth, they leave behind scars.

  • Maculopapular acne-like skin lesions (85%)

Observed in men, they mainly affect the neck.

  • Erythema nodosum (50%)

Applies to women. It appears on the lower limbs and heals with discoloration.

  • Pattern symptom

It is skin hypersensitivity, caused by even the slightest damage. The pattern of patterns is one of the criteria for the diagnosis of Behcet's disease. It can be caused by a needle puncture or an intradermal injection of isotonic sodium chloride solution. Within 24 hours, erythematous sclerosis develops, with subsequent accumulation of sterile purulent discharge in the center of the lesion. In Middle Eastern countries, this symptom may be considered pathognomonic for Behcet's disease.

  • Ocular symptoms (50%)

Patients complain of double vision, photophobia, redness, pain. There is uveitis and inflammation of the retina. Untreated eye ailments may lead to the development of glaucoma, cataracts or blindness in the future.

  • Arthritis (40%)

The disease process may involve one or more joints. Usually large joints are attacked, rarely small ones, and almost never sacroiliac joints. Inflammation can be acute or subacute. Patients suffer from swelling and pain in the joints making it difficult to move. Symptoms last up to three weeks and then disappear spontaneously. However, in some cases, destructive changes in joints occur.

  • Myositis

Only in individual cases was generalized myositis described. Some people experience local inflammation.

  • Vascular thrombosisvenous (24%)

It occurs in acute cases of the disease.

  • Vessel changes

The disease is systemic and can affect any vessel: small, medium and large. Clinical symptoms depend on the location and intensity of the inflammatory process. The arteries become constricted and aneurysms, while the veins develop thrombosis and varicose veins.

  • Heart involvement (7-30%)

May occur: conduction disturbances, pericarditis, as well as coronary vasculitis and symptoms of ischemic disease

  • Changes in the digestive tract

Usually affects the large intestine. Ulcers appear that may cause perforation and peritonitis.

  • CNS involvement (5%)

It appears at the latest, even a few years after the aphthous ulcer appears in the mouth. It is referred to by the term "neuro-Behçet". Symptoms include headache, fever, confusion, balance disorders, and strokes. Compared to the he althy population, they are more likely to suffer from aseptic meningitis.

  • Kidney involvement (3%)

Rare compared to other symptoms.

Behcet's disease: diagnosis

The diagnosis of the disease is based on the clinical picture, as there are no specific laboratory tests or histopathological picture. May appear: anemia and leukocytosis, acceleration of ESR, increased concentration of C-reactive protein, immunoglobulins (mainly IgA and components of the complement system), disorders in the coagulation system predisposing to thrombosis. However, no antinuclear antibodies or rheumatoid factor are present.

In 1990, a group of experts - International Study Group for Behçet's Disease - developed the criteria for the diagnosis of Behcet's disease: 1) main criterion: mouth ulcers - small, large or herpes-like, found at least 3 times within 12 months2) criteria additional:

  • changes in the organ of vision: anterior or posterior iritis, inflammation of the retinal vasculitis
  • positive allergy test (pattern symptom) - read after 24-48 hours
  • recurrent genital ulcers
  • erythema nodosum, follicular-like or folliculitis-like lesions, acne nodules in adults not treated with corticosteroids.

To establish the diagnosis of Behcet's disease, it is necessary to meet the (main) criterion and at least two additional criteria after excluding other causes.

Behcet's disease should be differentiated from:

  • Reiter's team
  • seronegative arthropathy
  • sarcoidosis
  • forms of systemic vasculitis

Behcet's disease: treatment

Due to the unclear etiology of the disease, causal treatment is not possible. All that remains is to alleviate the symptoms of the disease. The therapy used depends on the advancement of the disease and organ involvement.

Drugs used in therapy: corticosteroids, non-steroidal anti-inflammatory drugs, azathioprine, cyclosporine, sulfasalazine, colchicine, antiplatelet drugs, anticoagulants, cyclophosphamide, dapsone.

In cases limited to the skin, local application of drugs - corticosteroids, antibiotics is enough. In more severe cases, stronger drugs (cytostatics, immunosuppressants) are used.

Behcet's disease: prognosis

The disease has periods of exacerbation and remission. In cases where the CNS and lungs are not involved, the prognosis is good. The most common causes of death are neurological complications, aneurysm rupture and lower limb vascular thrombosis.

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Immune system