Behcet's disease in children

Introduction to Pediatrics Behcet's disease (Behcetsdisease, BD), Behcet's disease is an unexplained cause, which occurs in young men and young adults. It is characterized by oral ulcers, vulvar ulcers, ophthalmia and skin damage. Chronic diseases involving multiple systems. basic knowledge The proportion of illness: 0.001% Susceptible people: children Mode of infection: non-infectious Complications: epilepsy glaucoma corneal ulcer

Causes of Pediatrics Behcet's disease

(1) Causes of the disease

The exact cause is unknown, and the available data suggest that environmental and genetic factors are related to the occurrence and development of the disease.

Environmental factors

(1) Microbial infection: Herpes simplex virus, hepatitis C virus, streptococcus sanguis, tubercle bacilli are suspected as possible causes, but there is no definite evidence. Some people think that heat shock protein (HSP), a kind of eukaryotic The extremely conservative and widely-existing molecules that remain in the process of biological evolution, which can be produced by bacteria or host cells under the stimulation of heat, contact with free radicals, lack of oxygen, and lack of major nutrients. The HSP produced by bacteria can stimulate the patient's T. Lymphocytes.

(2) Geography: The population with high prevalence of the disease is located in a characteristic area, across Asia, which is the road of commercial exchange between East and West in the same year, and is also a Turk. The patients after migration to Germany are more than those of Europe. The Turks are five times lower, 18 times lower than the Turks in Asia, and the Japanese who migrated to Hawaii have a lower prevalence than Japanese natives, suggesting that an unknown geographically relevant factor is at work.

(3) Race: The prevalence of Western Caucasians is significantly lower than that of Middle Easterners and Yellows. The prevalence of Turkish in Iran is significantly higher than that of other ethnic groups in the country. Another ethnic minority in Iran comes from the ancient Family (white race), rare BD.

2. Genetic factors

(1) Family survey: BD has a family history of 2% to 3% in Japan, and 8% to 34% in Turkey and other Middle Eastern countries, most of which are first-degree relatives. Turkey has reported the risk of sickness of patients. The factor reached 11.4 to 52.5, indicating that BD has a genetic tendency.

(2) Genetics:

1HLA-B5 (B51): BD has been closely related to HLA-B5 (B51), with 81% of BD patients with this gene in Asia, 55% in Japan, and 10% to 15% in control population. Risk 6.7, the positive rate of HLA-B5 in BD in 10 countries in Europe is 25% to 79%, and the control group is 3% to 28%, the relative risk is 1.5 to 10.9, and HLA- in patients with BD in China. The B frequency is 37.5%, and many people believe that HLA-B*5101 (subtype of B5) has a determinant of the Behcet disease gene (ie DAIXXXXXF), which may act as an autoantigen, in the presence of severe visceral lesions and eye diseases. The positive rate of BD, HLA-B5 (B51) is higher than that of those without visceral lesions and eye diseases, so it is also considered to be related to the severity of BD disease.

2MIC: This gene is located between the HLA-B site of chromosome 6 and the TNF site, close to the B site, with subtypes A and B, MIC A and BD, and MIC A is significantly higher in BD than in control population ( The former is 74.0%, the latter is 45.6%), but some people think that MIC A has a strong linkage with B51, and its heterogeneity is low, so it is not like a major gene related to BD, MIC A is mainly composed of endothelial cells. And fibroblast expression, which stimulates the activation of T lymphocytes and NK cells, so vascular damage to BD may be related to it.

(two) pathogenesis

The pathogenesis of BD involves cellular and humoral immunity.

Cellular immunity

(1) Activated T cells appear in the local tissues and peripheral blood of patients, in which CD4 and CD8 are increased, T cells are also increased, and the T cell receptor TCR strain is inconsistent in each patient, that is, TCRV is polymorphic. It is suggested that the rise of T cells is caused by a variety of different antigens. Because IL-2 and IFN are increased in peripheral blood, the cytokines IL-4 and IL-10 secreted by Th2 are low, so BD is dominant in Th1. The cellular immune response, while the proinflammatory cytokines IL-1, TNF- and IL-8 in the blood circulation are also elevated.

(2) neutrophil response is a non-specific cellular response, which has a certain role in this disease, such as non-bacterial suppurative folliculitis, acupuncture reaction, anterior chamber empyema A large number of neutrophil infiltration, activation and hyperfunction, neutrophils from BD patients have the ability to produce a large number of peroxides and lysosomal enzymes and strengthen chemotaxis, resulting in tissue damage, neutrophils Activation may be associated with the induction of inflammatory cytokines.

(3) Endothelial cells: vascular endothelial cells lining the inner wall of blood vessels, providing a smooth surface for blood flow, maintaining the normal flow of blood, and also regulating the exchange of substances inside and outside the blood vessels as a permeable membrane. In recent years, it has been found to synthesize and release active substances. Such as vasodilation factor and contraction factor, anticoagulation and procoagulant factors, promote and inhibit vascular wall cell growth factor, prevent blood cells from adhering to vascular wall factors, etc., when stimulated (such as proinflammatory cytokines), endothelial cells express The increase of adhesion molecules is beneficial to the adhesion of platelets and leukocytes to their walls, the formation of blood clots, the removal of leukocytes, the release of mediators that cause tissue damage, and the expansion of tissue damage, antigen presentation after endothelial cell damage, promotion The role of inflammatory response, it is currently believed that endothelial cells are involved in the occurrence and development of systemic vasculitis. However, due to the heterogeneity of endothelial cells themselves, the morphology and function of endothelial cells of different sizes, types and organs are different, explaining different blood vessels. The damaged organs in the inflammation and the clinical manifestations are different.

2. Humoral immunity

Unlike other diffuse connective tissue diseases with known autoantibodies, BD has no correlation with antinuclear antibody profiles, anti-neutrophil cytoplasmic antibodies, and antiphospholipid antibodies. In recent years, anti-endothelial cells have been considered. Antibodies (AECA) are associated with vasculitis, which can occur in a variety of vasculitic lesions: arteritis in primary vasculitis, Kawasaki disease, Wegener's granulomatosis, microscopic polyangiitis Etc., lupus nephritis and dermatomyositis in secondary vasculitis, the positive rate in BD is 28%.

The target antigen of AECA is very different in each vasculitis: there are DNA, DNA-histone, ribosomal P protein in SLE, and systemic vasculitis with protease 3 or myeloperoxidase. The target antigen in BD is still unknown.

The causal relationship between AECA and endothelial cell injury is unclear, but it activates endothelial cells and promotes complement-dependent and/or antibody-mediated cytotoxic responses, leading to sustained or further progression of endothelial cell damage.

Pediatric Behcet Prevention

The key to preventive measures is to prevent the recurrence of diseases, pay attention to the combination of work and rest, maintain good mood; pay attention to cleanliness and prevent various infections; strengthen nutrition and improve the body's ability to resist diseases, can alleviate the disease. Pay attention to diet or mood in your life and cause recurrence of oral ulcers. You should seek medical advice promptly, follow the doctor's advice, and take regular medications for a long time to prevent the recurrence of the disease. Because the eyes may be blind after recurrent episodes of Behcet's disease, causing lifelong pain to the patient, so the main treatment for treatment is to treat Behcet's disease in order to protect the eyes. Protect eyesight.

Pediatric Behcet's disease complications Complications, epilepsy, glaucoma, corneal ulcer

Can cause blindness, corneal ulcer, retinal vasculitis, retrobulbar optic neuritis, retinal hemorrhage, vitreous opacity, glaucoma; nervous system damage can occur with disturbance of consciousness, mental disorders, epilepsy, sensory disturbances; cardiovascular disease can be moved, Venous obstruction, aneurysm and varicose veins, aortic involvement when the aorta is involved; ulceration, perforation can occur in the digestive tract; pulmonary complications such as bronchospasm or pulmonary infarction can occur.

Symptoms of Pediatric Behcet's disease Common symptoms Mouth, eye, genital damage Oral ulcers Joint swelling and swelling Follicular squamous keratitis Esophageal ulcer Muscle myasthenia erythema nodules Peripheral nerve damage Headache and vomiting

The whole system is involved, and the more common ones are the oral cavity, the skin, the genitals and the eyes, the central nervous system, the blood vessels and the digestive tract are all serious, and the male incidence is high. Japanese scholars report the incidence of various organ system damage. They were: oral ulcer 100%, rash 90%, external genital ulcer 97%, eye damage 79%, eye damage more men than women, the incidence rate was 90% and 40%, respectively, other system damage is the central nervous system 28%, digestive system 20%, cardiovascular system 15%, joint 15%, Huang Zhengji and other analysis of the incidence of clinical manifestations of Behcet's disease in China: 99% of oral ulcers, 43.2% of ocular lesions, 73.6 of external genital lesions %, skin lesions 96.8%, nervous system 26%, large blood vessels 8.7%, digestive tract 8.4%, arthritis 60.9%.

A small number of patients are acutely ill, and can be damaged at the same time in two or more parts within 5 days to 3 months. The condition is severe, often accompanied by high fever. After a certain period of remission, chronic recurrent episodes can occur. The time is 1 to 2 months, most of which are chronic diseases. The damage occurs in one part. After repeated episodes and remissions in different periods, the disease occurs in other parts. The latter is mainly local damage, and the systemic symptoms are less, but It can be acutely aggravated during the course of the disease.

Whether it is acute or acute exacerbation in a chronic course, the main systemic symptoms are high fever, headache, fatigue, loss of appetite, joint pain or swelling, etc., heat type is uncertain, a few cases can continue to be hot, excessive fatigue, poor sleep Menstruation, seasonal climate changes, etc., can make the damage of different parts worse, a small number of patients have a family history, no contact infection cases.

Basic symptoms

Refers to the most common and often the first symptoms in this disease, they can appear in succession or at the same time for several years, most of the onset is hidden, a small number of onset is accompanied by fever, fatigue and other systemic symptoms .

(1) recurrent oral ulceration (recurrent oral ulceration): at least 3 episodes per year, more than one painful red nodules appear in the buccal mucosa, lips, lips, soft palate, etc., followed by ulceration, the diameter of the ulcer is generally 2 ~ 3mm, some with herpes onset, about 7 to 14 days after the self subsided, leaving no scars, there are several weeks of unhealed scars, ulcers one after another, this symptom is seen in 98% of patients, and this is The first symptom of a disease, which is considered to be the most basic and necessary symptom for the diagnosis of this disease.

(2) recurrent genital ulceration (recurrent genital ulceration): basically similar to the symptoms of oral ulcers, but the number of occurrences is small, the number is also small, the common site is the female patient's large, labia minora, followed by the vagina; male scrotum And the penis can also appear around the perineum or anus, about 80% of this symptom.

(3) Skin lesions: Skin mucosal damage is a major symptom of this disease, accounting for 95.7%, and the following four types of skin damage:

1 nodular erythema: it is the most common type of skin damage, mainly in the lower limbs, especially the lower leg, sometimes in the upper limbs and trunk, more common in the extension of the side, the subcutaneous nodules are generally broad beans to walnuts, shallow Different, there are pain and tenderness, the skin color is reddish, dark red or purple, the texture is hard, ranging from a few to dozens, irregular distribution, about 1 month can be self-resolving, but easy to relapse, or For one side to fade, one side is new, most of which are heavier in summer, rarely ruptured, and some of the nodular red range can exceed the nodule area. The more outward the color, the lighter it seems to have a blush.

2 thrombophlebitis: mainly occurs in the lower limbs, sometimes also in the upper limbs, for the hard subcutaneous ropes of the touch, there is local tenderness, if it occurs in the deep subcutaneous, the physical examination only has subcutaneous sling; if it occurs in Subcutaneous superficial veins may have different degrees of redness on the skin surface and high skin wounds. Superficial thrombophlebitis may also be secondary to intravenous drug injection.

3 folliculitis-like and sputum-like lesions: more men than women, folliculitis-like damage more than sputum-like damage, the lesion can occur in any part of the body surface, more common in the head, face, chest and genitals, how much skin lesions Different, it can be repeated, with summer as the weight, the basal infiltration of these lesions is more significant, the top pustules are less, the surrounding redness is larger, accompanied by different degrees of induration, bacterial culture is negative, antibiotic treatment is invalid.

4 acupuncture reaction: injection (muscle, vein), acupuncture and other stab wound skin dermis, after 24 to 48 hours, can produce reddish papules in the acupuncture site, the center has pustules, and later gradually scarred, this reaction is called For the acupuncture reaction, it subsided in about 1 week, and the disease activity period was mostly positive. It has specific diagnostic significance, and sometimes the acupuncture reaction is negative. At this time, it is injected with normal saline, and pustules can be present in the acupuncture site after 24 hours. It must be emphasized that Behcet's disease cannot be ruled out even if the acupuncture reaction is negative.

(4) Eye damage: The main manifestation of eye disease is Behcet's disease, also known as eye white crosis. Its main features are transient, regressive exudative inflammation, which can be affected by various tissues of the eyeball. Inflammation, combined with membranous inflammation, scleritis and iridocyclitis with anterior chamber empyema, after recurrent episodes, mainly manifested as choroiditis, uveitis, retinitis and fundus hemorrhage, vitreous opacity, etc., with the disease Development can eventually lead to blindness, which can be unilateral or bilateral.

Combined with membranous inflammation, keratitis and scleritis are caused by vasculitis due to inflammatory chemical stimulation or other harmful factors, and the combination of membranous inflammation is also a recurrent episode. 5 days, can be accompanied by varying degrees of keratitis, long-term unhealed often cause corneal ulcers, and even corneal perforation, and sometimes combined with membranous inflammation, recurrent keratitis, secondary cataract can occur.

Typical iridocyclitis with anterior chamber empyema is rare, anterior chamber empyema is aseptic, cell components are neutrophils, containing a small amount of fibrin, white blood cells mainly from inflammatory iris, retinitis characteristics For acute recurrent episodes, manifested as optic disc redness, turbidity, retinal leukoplakia, hemorrhage, etc. Vitreous opacity is one of the main manifestations of ocular damage in this disease. Each episode can aggravate it. This change precedes the anterior chamber. Turbidity, vitreous opacity is caused by inflammatory exudation of surrounding capillaries. Exudates of radial capillaries around the nipple connected to the vitreous membrane easily enter the vitreous and stimulate the intima to form granuloma tissue. Similarly, The granuloma tissue is also formed around the choroidal junction of the posterior ciliary body, and the granuloma formed in the posterior choroid of the vitreous and ciliary body is the main cause of vitreous opacity.

2. System symptoms

In addition to the above basic clinical symptoms, some patients still have lesions of the organ system. These lesions are also caused by local vasculitis. Most of the systemic lesions appear after the basic symptoms.

(1) Gastrointestinal lesions: This type is also called gastrointestinal-Behcet's disease. It occurs in 10% to 15% of patients. Gastrointestinal symptoms have abdominal pain and frequency of right lower abdominal pain. Local tenderness and rebound tenderness, followed by nausea, vomiting, abdominal distension, anorexia, diarrhea, swallowing discomfort, etc., through the gastrointestinal X-ray examination, endoscopy and surgical exploration to see the basic lesion of the intestine is from the lower part of the esophagus Multiple ulcers, ileocecal area is the most affected part, 7 of the 15 cases of endoscopy have ulcers, followed by ascending colon, descending colon, stomach, esophagus, etc. In short, this lesion can occur In any part of the entire digestive tract from the mouth to the anus, severe cases with ulcer bleeding, intestinal paralysis, intestinal perforation, peritonitis, fistula formation, esophageal stricture and other complications can even die.

(2) Neurological diseases: This type is also called neuro-Berchette disease. It is found in 10% of patients. The number of men with this lesion is significantly higher than that of women (10:4), except for some cases after the occurrence of basic symptoms. From month to year, the clinical manifestations vary with the affected part. Any part of the brain and spinal cord can be damaged by small vasculitis (even in the same patient, the nervous system can be affected in multiple parts), so the clinical system of the patient's nervous system The performance was extremely diversified. Among the 14 cases admitted to Peking Union Medical College Hospital, 6 cases showed abnormal mental behavior, even aphasia, 2 cases with conscious disturbance and convulsions, 2 cases with nausea and vomiting, 2 cases with spinal cord lesions, limb numbness, hyperalgesia. Among them, 1 case had dysfunction of the bowel and bladder, 1 case had peripheral neuropathy in addition to cerebral infarction, and 1 case had no neurological symptoms.

Patients with complicated neurological diseases have frequent onset, and can be divided into meningitis, brain stem damage, benign intracranial hypertension, spinal cord damage, and peripheral nerve damage according to their symptoms. The symptoms of each type are listed as follows:

1 meningitis type: headache, disturbance of consciousness, mental disorders, optic disc edema, meningeal irritation, bilateral pyramidal tract signs, hemiplegia and so on.

2 brain stem damage type: dizziness, headache, tinnitus, disturbance of consciousness, nystagmus, Horner syndrome, cranial nerve palsy, difficulty swallowing, cyanosis, respiratory disorders, epilepsy, etc.

3 benign intracranial pressure increased: headache, vomiting.

4 spinal cord type: lower limbs weakness, numbness, sensory disturbance, different degrees of paraplegia, urinary retention, incontinence, pathological reflex positive.

5 Peripheral nerve type: limb weakness, numbness, peripheral sensory disturbance, muscle atrophy, low sacral reflex, etc., combined with peripheral neuropathy were significantly less than central neuropathy, only 1/10 of central lesions.

Cerebrospinal fluid abnormalities are increased intracranial pressure, about 80% have mild leukopenia, mononuclear cells or multinucleated cells each account for half, 33% to 65% have protein elevation, brain MRI has certain help in diagnosis, it can be seen Multiple parts of the brain tissue (frontal, occipital, temporal, cerebellum, basal) multiple infarction, a small number of patients with local bleeding, 1 case of angiography showed left common carotid artery, severe left stenosis, neuropathy The recurrence rate and mortality rate are very high. About 77% of the patients have remission after treatment but still have sequelae. The death occurs mostly within 1 to 2 years after the onset of the nervous system.

(3) vasculitis: This type is also called vascular-Behcet disease. This section refers to large and medium vasculitis. It is found in 10% of patients. The so-called large and medium vasculitis refers to large and medium arteritis and large parts of any part. Middle phlebitis, this type is more common in men, 21 cases of vascular-Behcet disease in Peking Union Medical College Hospital, 4 cases of arterial involvement, 15 cases of venous involvement, and 2 cases of arteriovenous involvement.

1 large arteritis: When the aorta and its branches appear inflammation of the blood vessel wall, the wall is first thickened, and then the thrombus formation eventually narrows the lumen, and the sustained inflammatory reaction causes the elastic fibers of the artery wall to be destroyed and lost. The toughness forms an adenoma-like local enlargement, and the nourishing blood vessels in the nutrient area also aggravate the lesions of the large vessel wall due to inflammation. The clinical manifestations of arterial stenosis have no pulse or weak vein on the affected side, and the blood pressure is low, or Nothing can be detected at the healthy side, local vascular murmurs (neck, abdomen, etc.), cerebral artery stenosis, dizziness, headache, severe syncope, renal hypertension in renal artery stenosis, myocardial involvement in coronary artery involvement Ischemia, even myocardial infarction, when there is an aortic aneurysm, local pulsatile mass, some patients with aortic root tumor-like expansion caused by aortic valve insufficiency, eventually leading to right heart failure, there are also pulmonary hypertension.

Moderate thrombophlebitis is more common in the extremities, especially in the lower extremities. Superficial phlebitis can be found by general physical examination, but swelling of the lower extremities caused by inflammatory embolism in deep veins depends on local angiography to confirm the diagnosis. Peking Union Medical College Hospital traces Six patients with Behcet's disease and large and medium phlebitis who were admitted to the hospital survived for more than five years except for one case of loss of follow-up, but they lost their labor.

The diagnosis of large and medium vasculitis depends on medical history and detailed physical examination, angiography, and Doppler examination is a reliable examination of the scope of diagnosis and involvement.

2 It is not uncommon for the heart of Behcet's disease to be affected. Aortic valve insufficiency may occur due to aortic root tumor-like dilatation, and right ventricular dysfunction may occur due to incomplete tricuspid atresia. In addition, there are also atrioventricular block. Myocardial damage, reports of pericardial effusion.

Pediatric Behcet's examination

Skin irritation test

Forearm flexion was injected intradermally with 0.1ml of normal saline. Red induration or small pustules larger than 2mm in diameter appeared in 48h. The small papules were positive, suggesting that neutrophil chemotaxis was enhanced, and the positive rate was about 40%.

2. Determination of C-reactive protein (CRP)

CRP increased before and after the onset of ocular inflammation, especially before the onset of the attack; CRP increased with the increase of neutrophil count, the positive rate of eye attack within 1 week reached 86.1%, so it is considered that the CRP pair is determined. Predicting the onset of ocular inflammation has a certain value.

3. Erythrocyte sedimentation rate and white blood cell classification

When the disease occurs, the erythrocyte sedimentation rate is significantly accelerated, and the neutrophil ratio is also significantly increased.

4. Pathological examination

The basic pathological changes of all affected organs are vasculitis, mostly exudative, a few proliferative, or both. Acute exudative lesions manifest as luminal congestion, thrombosis, wall and surrounding tissues. Fibrin-like degeneration, and neutrophil infiltration and red blood cell spillover, neutrophil nucleus often breaks into nuclear dust, there is obvious edema, cellulose exudation, abscess formation, proliferative pathology is no exception.

5. Serum fibrinolysis system and immunogenetics (small arm of chromosome 6) can be examined if necessary or necessary.

Auxiliary inspection

X-ray inspection

Gastrointestinal barium meal examination, patients with a course of less than 7 years, often with stomach, duodenal bulb and small intestine ulcer, ulcer perforation may occur in some cases, the small intestine may have different degrees of functional changes, showing intestinal lumen widening, Intestinal mucosal folds are thickened and segmented. Sometimes the jejunal wall of the jejunum is straight and sausage-like.

Patients with advanced disease over 10 years may have a wide polypoid filling defect in the stomach, small intestine and colon, which is obvious in the small intestine and colon, and may have proliferative unequal knots in the ileocecal and ascending colon. Nodular filling defects, mucosal folds disorder, pathological diagnosis of ulcerative gyrus, colitis.

Pulmonary manifestations are less common. If pulmonary vascular infarction or hemorrhage, it is often seen in the lung field or the hilar area with similar pneumonia. The density of lung metastases is increased by round or elliptical shadows, and the heart shadow can be enlarged. There is mild ventricular enlargement, and arachnoid adhesions can be found in myelography.

Angiography can detect segmental stenosis changes in different parts of the renal artery, mesenteric artery, and cerebral arteries.

2. ECG

Some patients may have ST segment and T wave changes, most of which have sinus tachycardia.

3. EEG

When the nervous system is involved, most of them show diffuse slow waves, but there are also many waveform changes without characteristic waveforms. Those with brainstem symptoms may have diffuse slow waves, and those without brainstem symptoms may appear mild to medium. Slow wave.

4. Acupuncture reaction

This is the only specific test with strong specificity in this disease. It is practiced by using a sterile intradermal needle to penetrate the skin in the middle of the flexion of the forearm and then withdrawing. After 48 hours, observe the skin reaction at the needle penetration. If there is red papules or red papules with white herpes, it is considered as a positive result. At the same time, some cases of positive acupuncture test, but some are negative, the patient is undergoing venipuncture examination or intramuscular When the treatment of injection, acupuncture positive reaction often occurs, and the positive rate of venipuncture is higher than that of intradermal puncture.

The positive reaction of acupuncture has no obvious relationship with the site of disease involvement, but it is positively correlated with the activity of the disease. The positive result of acupuncture test appears in more than 60% of BD patients in China, while the positive rate in the Mediterranean coastal countries is 80%. In Europe and the United States, the positive rate of this test is low, and the false positive of this test is less, and its specificity is 90%.

5. Other related inspections

According to the patient's clinical manifestations, related examinations of the affected system, including imaging such as magnetic resonance, angiography, ultrasound cardiovascular examination, endoscopy, cerebrospinal fluid, etc.

Diagnosis and diagnosis of Pediatrics

diagnosis

Behcet's International Diagnostic Standard

1. Repeated oral ulcers: Some doctors observed or patients complained of aphthous ulcers or scars, repeated attacks within 3 years 3 times.

2. Repeated genital ulcers: A doctor observed or the patient complained of genital aphthous ulcers, especially men.

3. Eye lesions: Before and/or after uveitis, there is cell infiltration or retinal vasculitis in the vitreous during slit lamp examination.

4. Skin lesions: nodular erythematous lesions, pseudo folliculitis, purulent papules, acne-like rash (without taking glucocorticoids).

5. Acupuncture reaction positive: With a 20-gauge sterile needle, obliquely into the skin, 24 to 48h by the doctor to determine the results.

Anyone with repeated oral ulcers accompanied by 2 of the remaining 4 items can be diagnosed as this disease.

Other symptoms that are closely related to the disease and are conducive to the diagnosis of this disease are: joint pain (arthritis), subcutaneous embolic phlebitis, deep vein thrombosis, arterial embolism and/or aneurysm, central neuropathy, digestive ulcer, Epididymitis and family history.

Although oral ulcers are not specific in nature, they occur in 98% of BD, and when it is combined with vulvar ulcers, eye lesions, and skin lesions, the specificity is greatly improved, so it is considered to be one of the basic symptoms of BD. When two symptoms are present in the three main symptoms of oral ulcer, external genital ulcer and eye damage, Behcet's disease can be diagnosed on the premise of excluding other related diseases.

Differential diagnosis

Systemic lupus erythematosus

There may be eye lesions, oral ulcers and nerves, cardiovascular system lesions, but the disease is progressively aggravated, not periodic episodes, and LE cells, anti-nuclear antibodies are positive, these abnormal findings are never seen in Behcet's disease.

2. Wegener granuloma

Although there are ocular lesions and multiple systemic injuries, the disease progresses progressively, and the lung X-ray examination shows a variety of infiltrates, sometimes with cavities, histopathological features of granulomatous vasculitis, and renal dysfunction. Severe, no genital ulcer, negative acupuncture test, it is easy to distinguish with Behcet's disease.

3. Tuberculous arthritis

Sometimes accompanied by nodular erythema, but no eye damage and genital ulcers, generally no cardiovascular and nervous system damage, anti-tuberculosis treatment is effective, although tuberculosis infection can cause Behcet's disease, effective against sputum treatment, but caused by Mycobacterium tuberculosis Behcet's disease not only has nodular erythema and arthritis, but also vascular system, nervous system and mucosal changes, the identification of the two is not difficult.

4. Multiple arteritis

When Behcet's disease is characterized by vascular lesions, it should be distinguished from multiple arteritis. The latter is mainly characterized by no pulse in the upper or lower limbs, no oral cavity, genital ulcer, and histopathological changes to giant cell arteritis. Vein changes, negative acupuncture reactions, and few skin lesions.

5. Crohn's disease

Intestinal Behcet's disease has similar clinical manifestations as Crohn's disease, but it should still be treated as two different diseases. Incomplete intestinal Behcet's disease and Crohn's disease are associated with oral cavity, perineal ulcer, clinical It is not easy to identify, and the two can be distinguished by histopathology and angiography.

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