What is Behcet's disease

Behcet's disease is Behcet's syndrome, a relatively rare disease. The scientific cause of Behcet's disease has not yet been clearly determined. It may be related to the living environment, one's own immune system, trace elements in the body, or genetic genes. Therefore, if you have Behcet's disease, you must seek medical attention in time and receive reasonable treatment, and try not to delay recovery.

The onset of Behcet's disease is also quite special. Generally, ulcers will occur in the oral or private parts. In addition, inflammation of the uvea of ​​the eye is also one of the symptoms. Therefore, Behcet's disease needs to attract enough attention from everyone. Once the disease occurs, we must pay more attention to all aspects of life. The following is a detailed introduction to the causes of Behcet's disease and various related contents. I hope it will be helpful to you.

Causes

The exact cause is unknown. The occurrence and development of this disease may be related to environmental, immune, trace elements, hormones and genetic factors.

Clinical manifestations

1. Basic symptoms

Oral ulcers, genital ulcers, uveitis.

2. Special symptoms

Skin lesions, gastrointestinal lesions, vascular lesions, lung lesions, arthritis, urinary system lesions, and nervous system lesions.

Because multiple systems of the body can be affected, the clinical symptoms and signs are complex and varied, mainly as follows:

(1) Oral ulcers About 98.6% of patients suffer from oral ulcers. 55.2% are the earliest onset symptoms of the disease, which may recur. It can occur in any part of the oral mucosa, tongue, and tonsils.

(2) Eye symptoms occur later and cause greater harm. The clinical manifestations are diverse, including recurrent keratitis, anterior chamber abscess, iridocyclitis, choroiditis, retinitis, optic neuritis, optic atrophy, conjunctivitis, etc. Eye damage can often lead to decreased vision or even blindness.

(3) Genital ulcers are most common in women with labia minora and labia majora ulcers, followed by the vestibule mucosa and around the vaginal opening. It sometimes occurs in the perineum and anus, and can also spread to the cervix. The number and size of ulcers vary, some are more numerous and shallower. The edges of the ulcer are sunken inward, the surrounding area is red and swollen, the ulcer is covered with some pus, and it often takes weeks to heal. Gangrenous ulcers are more serious, and patients may have fever, general discomfort, and local pain. Ulcers tend to be smaller and deeper over a few days, with irregular and inward-looking edges. The surrounding inflammation is obvious, and there is a dirty yellow or gray-black necrotic pseudomembrane on the surface. The false membrane is forcibly peeled off to reveal the uneven base. Sometimes the ulcer expands rapidly, forming a huge erosive ulcer, which leaves the labia minora incomplete, with soft edges and no infiltration. The pathological examination of the ulcer is non-specific.

(4) The most common skin symptom is erythema nodosum, but erythema multiforme and acne-like rash can also be seen. There is an allergic reaction to skin puncture. Such as impetigo, folliculitis, furuncle, cellulitis and ulcers. An erythema nodosum-like rash sometimes occurs. Small papules or pustules will appear when the skin is punctured with a sterile needle.

(5) The manifestations of the cardiovascular system are mainly allergic small vessel vasculitis, which may also include occlusive phlebitis, endarteritis, aortitis, aortic valve insufficiency, and peripheral artery aneurysms. The lesion affects veins, both small ones such as the retinal vessels and large ones such as the superior and inferior vena cava. Pulmonary thrombophlebitis may cause pulmonary infarction and repeated hemoptysis; multiple pulmonary artery thrombosis may cause cor pulmonale.

(6) Although neurological symptoms only account for 8% to 10%, the condition is serious and the most harmful, with recurrent paroxysmal headaches being the most common. Central nervous system thrombophlebitis and microangiitis can cause focal softening of brain tissue. Neurological symptoms appear later than other symptoms and may include dizziness, memory loss, severe headache, movement disorders, recurrent paraplegia and complete paralysis, and coma. Clinical manifestations include brainstem, meningeal, encephalitis syndrome and organic mental disorder syndrome.

(7) The incidence of gastrointestinal lesions is 50% to 64%, which can cause ulcers in the entire digestive tract and mucosa from the mouth to the anus, leading to perforation and proliferative changes. It can also cause Budd-Chiari syndrome due to hepatic vein occlusion. It is often misdiagnosed, increasing its harmfulness.

(8) Although the symptoms of hyperpyrexia and sepsis are mostly irregular low-grade fever, some cases present with remittent hyperthermia accompanied by leukocytosis, which is similar to sepsis.

(9) Joint and muscle symptoms: All joints of the limbs and the lumbar and sacral regions may be affected, with the knee joints being the most common. Symptoms include rheumatic pain without deformity or bone destruction. The knee, ankle and wrist joints are most commonly affected, with varying degrees of functional impairment, which can return to normal later.

diagnosis

The international diagnostic criteria for Behçet's disease are 1 necessary condition plus two of the four minor conditions.

1. Necessary conditions

Recurrent oral ulcers: at least 3 aphthous or herpetiform ulcers observed within a year. The disease presents with distinctive oral ulcers, which are located on the inside of the lips, gums, tip of the tongue and its edges, buccal mucosa, and soft palate mucosa. They can be single or multiple, usually 3 to 5, and range in size from a grain of rice to a soybean (3 to 15 mm). Round, oval or irregular in shape, with clear but irregular edges. The depth varies, there is yellow or white fur at the bottom of the ulcer, and a red halo can be seen around it. After the ulcer appears, it usually heals after 1 to 2 weeks. The disease often recurs, some occur continuously, and some do not occur for a long time. In severe cases, there may be throat ulcers, which are large and deep, with severe symptoms and slow healing.

2. Secondary conditions

(1) Recurrent genital ulcers with ulcers or scars observed currently or in the past.

(2) Anterior or posterior uveitis due to eye injury, or cells in the vitreous body or retinal vasculitis detected by an ophthalmologist using a slit lamp.

(3) Skin lesions: currently or in the past, there has been erythema nodosum or pseudofolliculitis, or purulent papules, or acne-like nodules (seen after puberty in patients who have not taken hormones).

(4) The doctor will determine whether the acupuncture test is positive within 24 to 48 hours.

complication

It can cause blindness, corneal ulcers, retinal vasculitis, retrobulbar neuritis, fundus hemorrhage, vitreous opacities, and glaucoma; damage to the nervous system can cause impaired consciousness, mental abnormalities, epilepsy, sensory disorders, etc.; cardiovascular diseases can lead to arterial and venous obstruction, aneurysms and varicose veins, and pulselessness when the large arteries are affected; ulcers and perforations may occur in the digestive tract; complications such as bronchial fistulas or pulmonary infarction may occur in the lungs.

treat

There is currently no effective cure for this disease. Although medicine cannot cure the disease, it can relieve symptoms and control the progression of the disease as long as you receive regular treatment. The main drugs used to treat this disease are:

1. Glucocorticoids

It can relieve various symptoms, especially improve mucosal ulcers and joint pain. For those with eye damage and central nervous system damage, larger doses should be used in a timely manner.

2. Immunosuppressants

It can prevent disease progression, have a synergistic effect with glucocorticoids, and reduce the dosage of glucocorticoids. Commonly used ones include cyclophosphamide, methotrexate, azathioprine, etc. In addition, there is cyclosporine A, which is effective for eye diseases, but symptoms are prone to relapse after discontinuation of the drug.

3. Anti-tuberculosis drugs

People with tuberculosis should actively receive anti-tuberculosis treatment. Some patients' condition improved after anti-tuberculosis treatment.

4. Nonsteroidal anti-inflammatory drugs

For example, aspirin has anti-platelet aggregation effect and can be used for people with thrombosis; other drugs such as ibuprofen, indomethacin, naproxen, Chinoly, and Voltaren can also be used, they are effective for joint pain and arthritis.

5. Other medications

For example, colchicine can inhibit leukocyte chemotaxis and reduce stimulation and inflammatory responses. Thalidomide has a good effect on mucosal ulcers.

The disease is generally not serious and is not life-threatening in most cases. In fact, a small number of patients may develop serious or fatal complications, such as central nervous system lesions such as meningoencephalitis, gastrointestinal perforation causing acute peritonitis, and large vessel lesions causing aortic aneurysms, which can be immediately fatal after rupture. If you have a disease, you must treat it. If you don't, the consequences will be serious.

Patients should pay attention to the following in their daily lives: lead a regular life, balance work and rest, and take proper rest when symptoms are significant. Avoid acupuncture whenever possible because it can cause inflammatory skin lesions. Eat less spicy food to protect the oral mucosa. Apply antibiotic eye ointment to your eyes before going to bed. Do not wear contact lenses to prevent corneal ulcers.

Keep the vulva clean and dry, reduce friction, etc.

Everyone knows about Behcet's disease. During the illness, patients should eat more light foods and avoid spicy foods to avoid affecting the condition and efficacy of the medicine. In addition, the skin of the affected area must be kept clean and dry. Do not touch the affected area with your hands to avoid cross infection.