Behcet’s disease is a chronic inflammatory disease that can invade various organs such as the oral cavity, genitals, eyes, skin, blood vessels, gastrointestinal tract, central nervous system, heart and lungs. The basic characteristic of each symptom is vasculitis, in which blood vessels are inflamed. Behcet’s disease tends to first start in the 20s and 30s, and the disease’s activity gradually declines over time after the onset.
Symptoms
Symptoms vary widely, such as ulcers in various organs, symptoms on the skin and eyes, arthritis, and invasion of other organs.
1) Mouth ulcers
It is the first symptom that appears in most patients. One or several may develop, starting with a slightly raised redness and gradually developing into an ulcer. It occurs repeatedly in any area such as the oral mucosa, tongue, gums, and lips, and the pain is severe, and most of them are healed without leaving a scar. Pain can interfere with food intake, and in severe cases, it can be accompanied by a feeling of weakness in the whole body.
2) Genital ulcers
It is similar to oral ulcers and tends to recur, but the frequency is much less than that of oral ulcers. It occurs in about 70% of patients, and usually occurs after oral ulcers have occurred. They are larger and deeper than oral ulcers, last longer and often heal with scarring. It occurs most often in the exogenous period in women and in the scrotum in men.
3) Skin symptoms
Nodular erythema in women, pseudofolliculitis (a kind of foreign body reaction to hair, local folliculitis, but not the primary infection), papulopustular (rash with pus) symptoms or acne-shaped nodules are common in men. Skin symptoms usually appear in the front of the lower extremities, but can also occur on the face, neck, upper extremities, and buttocks. Symptoms pass over several weeks. At first, a dark pigment is deposited in the area where the pathological change appears, then disappears without leaving a scar. Recurrence occurs depending on the activity of the disease.
4) Pathergy reactions
Pathergy reaction is a non-specific hypersensitivity reaction to skin irritation and can be seen not only in the skin but also in other areas. It can be easily tested using a disposable needle, and if papules or pustules develop after 24 to 48 hours, the pathergy reaction is said to be positive. If uveitis worsens after cataract surgery or ulcers at the surgical site after intestinal surgery are complications, it is a kind of pathergy reaction.
5) Eye symptoms
Pathological symptoms occur in the uveum and retina, and without active treatment, complications that impair vision may occur or blindness may occur.
6) Arthritis
It occurs well in the knees, ankles, and hand joints, most of them are temporary, and deformation or bone destruction is not common.
7) Invasion of other organs
In patients with Behcet’s disease, ulcers of the gastrointestinal tract are relatively common, and symptoms such as abdominal pain, diarrhea, and bloody stool appear. In addition, various vasculitis and diseases of the central nervous system also appear. In some cases, invasion of the central nervous system may appear before other symptoms of Behcet’s disease. In addition, invasion of the lungs and kidneys may also occur.
Causes
The exact cause of Behçet’s disease has not been identified, but it is thought that for a long time in patients with a genetic predisposition, the immune response is activated by the addition of environmental factors, and as a result, various symptoms appear. In particular, the gene HLA-B51 is known to be the most important genetic factor in Behcet’s disease, and it is known that there is a large difference depending on the region. It is also possible that the immune response to some viruses and bacteria is related to the inflammatory response of Behcet’s disease.
Diagnosis methods
Since Behcet’s disease has no symptoms or test results that are conclusively helpful for its diagnosis, a classification criterion consisting of those with high diagnostic value among the various clinical symptoms of Behçet’s disease is used.
Test methods
Tests for Behcet’s disease include:
1) Gene (HLA-B51) test
Genetic testing is only a reference for diagnosis, and clinical symptoms are the actual diagnostic criteria.
2) Pathergy reaction test
This test uses a disposable syringe to cause a wastewater reaction to be observed. Pathergy reaction is positive if swollen papules or pustules occur at the test site after 24 to 48 hours.
Treatments
Since the cause of Behçet’s disease has not been clearly identified so far, the aim of treatment is to improve the quality of life by controlling individual symptoms, and to prevent irreversible damage to the eyes, central nervous system, blood vessels, etc. In the course of treatment, it is common to use drugs in combination, in order to maximize the therapeutic effect and minimize side effects. Depending on the symptoms, the following treatments can be performed.
1) Treatment for skin mucosa symptoms
It is helpful to the patient to apply the steroid ointment to be applied to the affected area. Colchicine is known to be effective in healing or preventing recurrence of oral ulcers. If the skin mucosa symptoms are not well controlled with these drugs, a small dose of steroids may be taken, or drugs such as azathioprine and interferon may be considered.
2) Treatment for arthritis
Nonsteroidal anti-inflammatory drugs, colchicine, etc. are used.
3) Treatment for eye symptoms
Uveitis can be broadly divided into anterior uveitis and posterior uveitis. In the case of anterior uveitis, topical use of mydriatics or steroids can improve, but in the case of posterior uveitis or retinal vasculitis, active treatment such as administering an immunosuppressant is essential to prevent vision loss. In this case, cyclosporine and azathioprine are used as immunosuppressants.
4) Treatment for other pathological symptoms
Gastrointestinal ulcers, unlike oral ulcers, do not respond well to treatment and are often accompanied by intestinal bleeding or perforation. Sulfasalazine and steroids are commonly used drugs, and surgical treatment is sometimes required. For central nervous system invasion, high doses of steroids and immunosuppressants are used.
Progress and complications
The clinical symptoms of Behçet’s disease are diverse, and the severity of the disease varies from only mild symptoms such as skin mucosal symptoms or arthritis to severe uveitis or severe sequelae caused by invading major organs such as the brain, lungs, heart, and kidneys. Although deterioration and improvement are repeated in most patients, in general, the severity tends to decrease over time, and the most severe course is seen in young male patients.
Prevention methods
Since invasion of major organs such as the eyes and the central nervous system can leave serious sequelae, it is necessary to seek early treatment from specialists such as rheumatology, ophthalmology, dermatology, neurology, obstetrics and gynecology depending on the site of the invasion.
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