Behcet's syndrome
Introduction
Introduction to Baisai Syndrome Behcet's disease is closely related to recurrent oral ulcers, and recurrent aphthous ulcers can occur in 90%-100% of patients. However, it not only invades the eyes, mouth and genitals, but vascular inflammatory diseases that can affect multiple systems throughout the body, except that the time of occurrence of various system and organ lesions is different. Some patients have lesions of 1-2 kinds of organs first, and later there are lesions of other organs, which makes it difficult to diagnose. basic knowledge The proportion of sickness: 0.2% Susceptible people: no specific people Mode of infection: non-infectious Complications: hemoptysis
Cause
Cause of Behcet's syndrome
The cause of Behcet's disease is not yet clear. Some people think that it is caused by viral infection or allergic reaction to infectious factors, but it has not been fully proved. Recently, there have been many studies in immunology, and it has been found that some patients have elevated serum immunoglobulins and immune complexes, and have various antibodies, such as anti-oral mucosal antibodies and anti-arterial wall antibodies. Therefore, some people think that this disease may be some factors, including an autoimmune disease induced by the virus.
Prevention
Behcet's syndrome prevention
The disease recurrent and progressive development has been for 40 years, and pulmonary vasculitis caused by hemoptysis or central nervous system lesions have a poor prognosis.
Complication
Behcet's syndrome complications Complications hemoptysis
It has been found that the disease also invades many organs, thus causing attention to its complications, first of all skin complications, almost all patients have skin nodular erythema, folliculitis, rash or pustular rash, but also concurrent Non-specific allergic reaction of the skin: If a sterile needle is used to penetrate the sterilized skin, it is characterized by nodules, induration, papules or small pustules. Other complications are not high, but they are all "fatal injuries", such as heart. Allergic small vasculitis of the vascular system, superior vena cava syndrome, endarteritis, aortic regurgitation, peripheral aneurysm, etc.; brainstem syndrome of the nervous system, recurrent paroxysmal headache, etc.; gastrointestinal system Mucosal ulcers, perforation, proliferation, etc.; hyperthermia-like manifestations; limb-scale joint rheumatism-like pain; large hemoptysis of the respiratory system, pulmonary vasculitis, etc., as long as these complications are available, the patient may be required Life, so be contemptuous.
Symptom
Symptoms of Behcet's syndrome Common symptoms Redness, abdominal pain, erythematous rash, diarrhea, repeated genital ulcer, acne-like rash, ataxia
Main symptoms: Complete type: 4 major subjects in the clinical process
1. Oral mucosal abdominal aphthous ulcer incomplete type: 3 main signs or typical eye symptoms plus 1 main sign
2. Suspected type of skin symptoms: 2 major signs appear in clinical course
(1) Nodular erythematous rash is only possible: repeated major episodes
(2) Subcutaneous thrombophlebitis
(3) Hair follicle-like acne-like rash
(4) Positive skin acupuncture test
3. Eye symptoms
(1) iridocyclitis
(2) Retinal choroiditis
(3) Secondary lesions of the first two
4. Vulvar lesions
(1) male scrotum aphthous ulcer
(2) female size labia vagina wall pain ulcer
Secondary condition:
Rheumatoid arthritis
2. Gastrointestinal symptoms, such as multiple ulcers in the ileocecal area
3. Epididymitis
4. Vascular system symptoms, such as occlusive thrombophlebitis, arterial occlusion, aneurysm, etc.
5. Nervous system symptoms
1. Repeated oral ulcers are observed by the physician or the patient is sure to complain of aphthous or herpetic ulcers, repeated 3 times in 1 year.
2. Repeated genital ulcers are observed by the physician or the patient affirms that the genitals have aphthous ulcers or cramps, especially men.
3. Eye disease uveitis (pre and/or post), cells visible in the vitreous during slit lamp examination, retinal vasculitis.
4. Skin lesions nodules erythematous lesions, pseudo folliculitis, purulent papules, acne-like rash (not seen with prednisone).
5. Acupuncture test positive with sterile 20 small needle, oblique into the thorn skin, after 24 to 48 hours, the doctor judged the results.
Anyone with repeated oral ulcers and more than 2 of the remaining 4 can diagnose the disease.
Other symptoms closely related to the disease and conducive to the diagnosis of this disease include joint pain (arthritis), subcutaneous embolic phlebitis, deep vein thrombosis, arterial embolism and / or aneurysm, central neuropathy, digestive ulcer, and epididymis Yan and family history.
Mainly manifested as oral ulcers, genital ulcers and ocular mucositis (triad), may have skin damage (quadruple), can also invade organs of other systems.
First, oral ulcers
The ulcer has many clear edges and pains. It is located on the lips, gums, tongue or buccal mucosa. The ulcer is round or oval. The surface has white or yellow pseudomembrane. It is often multiple, usually healed after 1-2 weeks, but recurrent. Some patients have persistent ulcers that affect appetite, and almost all patients have oral ulcers.
Second, genital ulcer
Most occur in the scrotum, glans, female labia, vaginal wall, and even the cervix, urethra, ulcer morphology and oral ulcers, but repeated does not resemble oral ulcers, physical examination can be seen after the vulva ulcer or ulcer healing scar.
Third, eye inflammation
Early manifestations of conjunctivitis, iridocyclitis, late anterior chamber empyema, ocular uveitis, conjunctiva, corneal and retinal hemorrhage, ocular inflammation is the first symptom of the disease, and more often in oral ulcers for several years After the occurrence, manifested as eye and periorbital pain, photophobia, but also pupillary deformation, side or bilateral vision is affected, this disease is the main cause of blindness in Japan, bleeding can be seen in the fundus, arteriovenous occlusion, optic atrophy.
Fourth, skin symptoms
Mostly nodular erythema and polymorphous erythema, folliculitis, acne-like rash and skin allergy, acupuncture test (Pathergytest), using a 20 or smaller needle, obliquely penetrate the skin under sterile conditions, 24 to 48 hours later Pustules or folliculitis appear in the punctured area, peripheral blush is positive, the positive rate in patients in Japan and Turkey is 90%, and in Chinese patients is 62.2%. The test is highly specific and rarely positive in other diseases or normal people. .
Fifth, joint performance
50% to 60% of patients complain of joint pain, acute or chronic synovitis can occur, but joint swelling and bone destruction are rare, and the large joints of the extremities, especially the knee joint, are most affected.
Sixth, central nervous system performance
10% to 18% of patients with central nervous system damage, can have headache, neck stiffness, meningitis, epilepsy, soft palate, sensory and dyskinesia, cerebellar ataxia, cranial nerve damage and various psychiatric symptoms, central nervous system Suffering more often indicates that the condition is serious and the prognosis is poor.
Seven, intravenous and arteritis
Almost different parts of the large, medium and small arteries and veins can be affected, but more common are embolism phlebitis of the lower extremities, varicose veins are not uncommon, often upper and lower vena cava obstruction, arterial involvement is mainly arterial occlusion and aneurysm, and Caused by finger necrosis, no pulse, aneurysm rupture, etc., pulmonary artery involvement caused by pulmonary hypertension.
Eight, gastrointestinal performance
Intestinal ulcers are more common in ileocecal areas, but also in other areas, including abdominal pain, diarrhea, hemorrhage, perforation, intestinal fistula, and intestinal stricture.
Nine, other
There may be sub-testosteritis, lung parenchyma may occur in the lungs; focal nephritis may occur in the kidney, several cases of leukemia have been reported in the country, and 2 cases have malignant histiocytosis, which is a malignant development of the disease. It is still the manifestation of Behcet's syndrome caused by these malignant diseases or the combination of the two diseases remains to be further studied.
Examine
Examination of Behcet's syndrome
During the active period of the disease, "erythrocyte sedimentation rate", mucin, sialic acid, alpha, globulin increased, some patients were positive for ceruloplasmin, and a few cases were positive for cold globulin. The microcirculation of the tip of the tongue shows that the nipple nipple is atrophy. This point has the value of auxiliary diagnosis. It can be checked for the pathogenesis of the organs, such as electrocardiogram, electroencephalogram, CSF, CT, MRI, X-ray film, gastroscope and colonoscopy. , angiography, Doppler ultrasound, etc.
Diagnosis
Diagnostic identification
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
1, acute female vaginal ulcer
For acute painful ulcers that occur in female yin, unmarried women are more common. There are three types of ulcers: gangrene, sexually transmitted diseases, miliary, gangrene, and high fever. Systemic symptoms, malignant and miliary Symptoms, subjective symptoms of ulcers are burning and pain, may be associated with lower extremity nodules erythema and oral ulcers, so some people think that it is the female genital manifestation of self-suppression, the course of disease is usually several weeks, often recurrence.
The disease has no history of sexual intercourse, ulcer pain, soft and repeated attacks can be distinguished from sexually transmitted diseases such as hard chancre and soft chancre.
2, genital herpes
Male herpes occurs in the penis, glans, coronal sulcus, etc., rarely involving the scrotum, females are common in the labia, clitoris, perianal and vaginal and even the cervix, often with a burning sensation, clustering small leeches soon, rupture erosion after a few days It forms a shallow ulcer, the base is soft, the course is short, and it is easy to relapse. Pregnant women with genital herpes can easily lead to miscarriage, premature birth, and can also infect newborns.
This disease forms a sore after herpes, soft, short course and other characteristics can be distinguished from Behcet's disease, hard chancre, acute female yin ulcer.
3, soft squat
The disease is one of sexually transmitted diseases, caused by Haemophilus ducrei, multiple painful soft ulcers in the genital area, round or oval, irregular edges can be jagged, uneven basal surface, pale yellow purulent secretion on the surface Things, easy to bleed.
4, hard squat
It is characterized by a single or a few painless hard ulcers in the genital area, no purulent, clear and tidy borders, swollen lymph nodes nearby, healing in 2-3 weeks, generally occurring in male glans, coronary sulcus, penis, female size labia, vagina Etc. Lips, tongue, anus, fingers are also good parts.
