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Yibian
 Shen Yaozi 
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diseaseBehçet's Disease
aliasBehcet's Disease
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bubble_chart Overview

Behcet's disease is a chronic progressive and damaging condition of unknown cause, characterized by recurrent episodes and multiple system involvement, with small vasculitis as its pathological basis. The oral cavity, skin, genitals, eyes, and joints are commonly affected areas, usually with milder symptoms; while the heart, large blood vessels, digestive tract, and nervous system are less frequently involved, often presenting with more severe conditions. The clinical manifestations are diverse, and the prognosis depends on the affected organs.

bubble_chart Etiology

Evidence suggests that immune abnormalities play a significant role in the pathogenesis of Behcet's disease, but its **disease cause** and **mechanism of disease** remain incompletely elucidated.

  1. **Infection**
    1. **Viruses**: Early theories proposed viruses as the cause, but subsequent epidemiological studies, tissue cultures, serological tests, animal inoculations, immunofluorescence, and electron microscopy failed to provide further confirmation. Some reports suggest that the disease may be related to autoimmune abnormalities triggered by chronic viral infections. Other studies have found evidence linking HSV-1 to the disease, such as elevated anti-HSV-1 antibody titers in patients' blood, HSV-1's impact on CD4 lymphocytes leading to immune dysfunction, and the presence of HSV-1 DNA homologous to that of peripheral **blood stranguria** lymphocytes in affected individuals.
    2. **Streptococci**: Due to the frequent occurrence of tonsillitis, pharyngitis, and periodontitis in some patients, it has been hypothesized that the disease is associated with bacteria from these foci. Studies have shown elevated anti-streptococcal antibody titers in patients' sera, with streptococci—particularly *Streptococcus sanguinis*—being the most closely related strains isolated from patients' oral cavities. Intradermal tests and macrophage migration inhibition tests using streptococcal components have yielded positive results. Additionally, the 65-KDa heat shock protein from streptococci can induce skin hypersensitivity and systemic symptoms. These studies, primarily conducted by Japanese researchers, suggest an important role in pathogenesis but have not reached a definitive consensus.
    3. **Subcutaneous node bacteria**: Since 1964, case reports in China have proposed a link between Behcet's disease and **subcutaneous node** bacterial infections. Some patients had **subcutaneous node** diseases—such as pulmonary **subcutaneous node**, lymphatic **subcutaneous node**, and other **subcutaneous node** foci—before the onset of Behcet's lesions. These could be old **sexually transmitted disease** foci, though active **sexually transmitted disease** foci were more common. Most OT tests were strongly positive. Anti-**subcutaneous node** drug therapy not only improved the primary lesions but also alleviated Behcet's-related damage, suggesting an allergic reaction to **subcutaneous node** bacteria. The 65-KDa heat shock protein from **subcutaneous node** bacteria has also been implicated in the disease.
  2. **Trace elements**: A few reports have noted elevated levels of trace elements—such as organochlorines, organophosphates, and copper ions—in affected tissues, including vascular endothelial cells, macrophages, sural nerves, aqueous humor, serum, and neutrophils. Copper ions were the most abundant, possibly due to occupational or environmental factors.
  3. **Genetic factors**: The disease exhibits regional clustering, such as higher prevalence in Mediterranean countries. Familial cases spanning 2–4 generations have been reported, with a male predominance. HLA-B5 (+) serves as an immunogenetic marker, with a positivity rate of 67–88%, indicating an association with HLA-B5. HLA-D, particularly HLA-DR, also shows some correlation. The susceptibility gene for Behcet's disease is located on the short arm of chromosome 6, between the HLA-B and TNF-beta loci, potentially guiding future gene therapy research. The disease lacks a clear inheritance pattern but may follow an autosomal recessive trait.
  4. Immune abnormalities: The patient's serum contains anti-oral mucous membrane antibodies and anti-stirred pulse wall antibodies; in addition, immune complexes are present in the serum, with a positivity rate of up to 60%, which is related to disease activity. Apart from elevated levels of IgG, IgT, and IgM grade I, IgE is sometimes elevated. DIF examination reveals the presence of IgG, IgA, CIC, and C3 in the vascular walls, particularly in the venule walls. In vitro tests show that the lymphocyte transformation test values in these patients are generally low, the DNCB skin test is mostly negative, and both T-cell and TH cell values are decreased. The infiltrating cells in erythema nodosum-like lesions are mainly T cells, particularly TH and NK cells, while the infiltrating cells in ocular tissues are primarily CD4 lymphocytes and macrophages, with few B cells and NK cells. These CD4 lymphocytes and macrophages are HLA-DR(+). The above findings indicate that this disease involves both humoral and cellular immune abnormalities, but the general consensus is that cellular immune abnormalities are more closely related to the pathogenesis of this disease.
  5. Others: Although the disease cause of this illness is not yet clear, its onset is related to immune abnormalities. During the processes of immune regulation and inflammatory responses, cellular components undergo changes and produce various active substances, such as plasmin inhibitors, which can reduce the activity of plasmin in dissolving fibrin, leading to increased fibrinogen levels; enhanced chemotaxis of neutrophils; and the production of tumor necrosis factor (TNF-beta), IL-2, and IL-6. These factors may also play a role in the progression of the disease.
  6. Sex hormones: Sex hormones such as testosterone, progesterone, estradiol, luteinizing hormone, follicle-stimulating hormone, and pituitary prolactin were measured in 21 males and preovulatory females. Statistical analysis showed that testosterone levels in males and progesterone levels in females were lower than those in the control group, with p-values of <0.01 and 0.05, respectively, while the rest were within the normal range. Additionally, PGF-2d levels were measured in 14 preovulatory female patients, with the average value being more than twice as low as that of the control group (p < 0.001).

bubble_chart Pathological Changes

The fundamental pathological change of this disease is vasculitis, involving capillaries, small and minute veins, with a few being small arteries. Among the larger blood vessels, veins are more commonly affected than arteries. The severity of lesions varies across different layers of the blood vessels. Generally, there is endothelial cell swelling and proliferation, with marked cases showing clustering into masses, along with vessel wall edema, slight deposition of eosinophilic material, separation of the muscle layer, thickening of the vessel wall, narrowing of the lumen, and rare thrombus formation. In small arteries, there is subendothelial fibrous proliferation and thickening of the intima. The vasa vasorum also exhibit the aforementioned changes. The vessel wall and surrounding tissues are predominantly infiltrated by lymphocytes, accompanied by extravasation of red blood cells and exudation of neutrophils. In skin tissues, the latter manifests as abscess-like formations without karyorrhexis. In cases with strongly positive OT tests, endothelial cell proliferation is often more pronounced, whereas in cases with positive pathergy tests and negative OT tests, neutrophil exudation is more prominent. Folliculitis-like lesions primarily consist of perifolliculitis with abscess formation.

bubble_chart Clinical Manifestations

In our country's cases, the disease is more common in women, with a male-to-female ratio of about 3:4. The age of onset ranges from 4 to 70 years, but mainly affects young adults aged 16 to 40. Many organs throughout the body can be involved, with the most common sites being the mouth, skin, genitals, eyes, and joints; the heart and large blood vessels, digestive tract, and nervous system are less frequently affected. The initial sites of lesions are: mouth (55.2%), skin (23.8%), genitals and eyes (6.75% each), with occasional reports of initial onset in the central nervous system. The disease manifests in two types: acute and chronic. A minority of cases present acutely, with lesions appearing simultaneously or sequentially in the mouth, skin, genitals, and eyes within 5 days to 3 months, accompanied by severe local lesions and systemic symptoms. The majority of cases are chronic, where lesions appear in one organ, followed by recurrent episodes and remissions over varying periods before affecting other organs. Typically, the mouth is affected first, followed by the eyes, with local lesions being predominant and systemic symptoms milder, though acute exacerbations can occur during the course of the disease.

Whether in acute onset or acute exacerbation during the chronic course, systemic symptoms include high fever, headache, lack of strength, loss of appetite, and joint pain. The fever pattern is variable, with persistent high fever being rare. 57.6% of cases experience low-grade fever in summer. Overexertion, poor sleep, around menstruation, sudden climate changes, or seasonal shifts can exacerbate lesions in different areas. A few patients have a family history, but no contact with pestilence cases has been reported.

(1) Oral lesions: Primarily painful ulcers, occurring in 99–100% of cases, are the earliest, most frequent, and longest-lasting type of lesion. They commonly appear on the tip and sides of the tongue, the inner side of the lower or upper lip, the lower or upper gums, and the buccal mucosa, while the palate is rarely affected. Lesions may be single or appear continuously, usually numbering 3 to 5. They begin as small, palpable "nodules" that quickly develop into ulcers. Ranging in size from a grain of rice to a mung bean, they are round or irregular, with clear but uneven edges, varying depths, and sometimes a pale yellow covering at the base, surrounded by a red halo. A single lesion typically heals in about 2 weeks. Early on, episodes occur about once a month; as the disease progresses, the intervals between episodes shorten, with lesions appearing continuously in a relapsing-remitting pattern, and long-term remission is rare. When the throat is affected, ulcers are fewer but deeper and larger, with severe symptoms and slow healing.

(2) Various types of skin lesions: Skin lesions are polymorphic, with an overall incidence of 97.4%, appearing relatively early and presenting as the initial symptom in 24.5% of cases.

1. Common and characteristic lesions:

(1) Erythema nodosum-like lesions: The most common type of skin lesion, accounting for 68.64%. Mainly seen on the lower limbs, especially the calves, sometimes on the upper limbs, and occasionally on the trunk and face. Generally about the size of a broad bean, moderately firm, skin-colored, pale red, bright red, or purplish-red, usually numbering a few to over a dozen, scattered irregularly. Grade I pain and tenderness, with no tendency to spread, merge, or form ulcers. A single lesion resolves in about a month, leaving a grade I pigmentation spot without skin depression. New lesions appear in other areas, and the same patient often exhibits lesions of varying sizes, depths, colors, and stages. In 32.37% of cases, newly emerging subcutaneous nodular lesions are surrounded by a bright red halo about 1–1.5 cm wide, known as the "halo phenomenon," a characteristic feature of this disease with high diagnostic value. The onset shows no clear seasonal pattern, though symptoms are generally more severe in summer.

(2) Folliculitis-like lesions: accounting for 41.15%. There are two manifestations. One is dark red papules the size of rice grains to mung beans, with or without small pustules at the apex, usually not ulcerating, and mostly disappearing after one week. The number is small, mainly distributed on the head, face, and upper chest and back. The other is pustular nodular lesions, which are more numerous and distributed on the thighs, calves, back, face, scalp, chest, forearms, buttocks, external genitalia, and perianal area. Initially appearing as red papules, they later develop rice-sized pustules at the apex, but without hair penetration. The base presents as an infiltrative induration with a wide surrounding erythema, causing grade I pain. Most do not ulcerate, with the erythema disappearing after about a week and the pustules being absorbed, while the infiltrative induration resolves more slowly. This manifestation is quite distinctive and holds significant auxiliary diagnostic value.

⑶ Pathergy reaction: Accounts for 64.4%. This is a unique skin reaction induced by external factors. It occurs the next day after the dermis is injured by intramuscular, subcutaneous, or intravenous injections, acupuncture, or other punctures, manifesting as foxtail millet-sized red papules or pustules. In severe cases, surrounding erythema and small nodules at the base may also be observed. This phenomenon can also occur after conjunctival injections, and venous wall injury may lead to localized phlebitis. The reaction typically subsides within 3–7 days. In severe cases, the reaction has a higher positive rate and intensity, whereas during remission, the positive rate is lower and the reaction milder. After the condition stabilizes, it may turn negative. This is a distinctive manifestation of the disease and holds significant auxiliary diagnostic value.

2. Less common but relatively typical lesions

⑴ Sweet’s disease-like skin lesions: Account for 2.41%. Lesions are distributed on the head, face, neck, forearms, dorsum of hands, lower legs, and dorsum of feet. They present as edematous erythema, red edematous papules and patches, and raised nodules, often accompanied by varying degrees of cutaneous pruritus. These lesions usually appear after oral lesions and may cease while other lesions continue to recur.

⑵ Superficial migratory thrombophlebitis: Accounts for only 3.38% of skin lesions and is more common in males. It primarily affects one or both great saphenous veins, with a few cases involving the dorsal foot veins or elbow veins, presenting as migratory thrombophlebitis. This condition occurs during recurrent and severe phases of the disease, with 61% of cases accompanied by varying degrees of fundus lesions. After 3–5 years of recurrent episodes, it tends to gradually subside and eventually stop.

3. Rare general lesions These mainly include erythema multiforme-like lesions, papulonecrotic subcutaneous nodules resembling bacterialid eruptions, annular erythema-like lesions, and pigmented purpuric lichenoid dermatitis-like lesions. Although these lesions resemble their respective independent diseases, their underlying pathology is related to superficial vascular changes in the skin. They appear later, have a lower incidence, and typically recur over 3–5 years. Their value in prognosis estimation and auxiliary diagnosis is limited.

(三) Genital lesions Primarily ulcers, accounting for 64–88%. They generally appear later than oral ulcers. In addition to occurring on the glans penis, vagina, labia majora or minora, and urethral orifice, genital ulcers are larger and deeper, fewer in number, more painful, and heal more slowly. They recur less frequently, with longer intervals between episodes—sometimes years apart—and may leave atrophic scars after healing. Rare complications include necrosis and rupture of scrotal veins and massive bleeding from vaginal ulcers.

(四) Ocular lesions These usually appear later, with an incidence of 43.0–75.6%. One eye is typically affected first before involving the contralateral eye. Both anterior and posterior segments of the eye can be the initial site of involvement, though the posterior segment is usually affected first. Even if the anterior segment is involved initially, the posterior segment will eventually be affected. Early lesions are relatively simple, presenting as keratitis or corneal ulcers, herpetic conjunctivitis, scleritis, choroiditis, uveitis, retinitis, optic papillitis, retinal arteritis or phlebitis, retrobulbar neuritis, or fundus exudates. Although iridocyclitis with hypopyon is relatively characteristic, it is not common. Early exudative lesions may resolve, but repeated episodes lead to more complex tissue damage. Severe cases may progress to phthisis bulbi or impair vision due to fundus hemorrhage, vitreous opacity, or secondary glaucoma. Uveitis can also cause blindness, accounting for 20–30% of blindness cases in Japan.

(5) Joint damage accounts for about 60%. Large and small joints of the limbs can be affected, either singly or multiply, symmetrically or asymmetrically, usually involving the large joints of the limbs, with the knees being particularly common, accounting for about 83%. The most common manifestation is pain, with redness and swelling being rare. Cold weather exacerbates the pain, but it is generally tolerable. Despite long-term recurrent episodes, the symptoms can resolve spontaneously. X-rays of the bones and joints typically show no significant abnormalities, and any damage is usually mild. Synovial membrane biopsies reveal only grade I lesions in the superficial layer. Notably, a few cases present with significant swelling in one or both fingers, elbows, knees, or other joints, either singly or multiply, with limited mobility but no redness. Fluid cultures are negative, and white blood cell counts are elevated. Conventional anti-inflammatory drugs are often ineffective, though spontaneous resolution occasionally occurs. Rare cases involve destruction of the calcaneus and metatarsophalangeal joints, cystic changes in the phalanges, or ankylosing spondylitis.

(6) Heart and Large Vessel Damage Cardiac damage is relatively rare, reported in about 6% of cases. Clinical manifestations include arrhythmias and changes in heart sounds. Autopsy findings often reveal cardiac hypertrophy, with less common occurrences of endocarditis, pericardial effusion, myocardial fibrosis, aortic valve insufficiency, mitral stenosis, tricuspid fibrosis, aortic sclerosis, and pancarditis.

Medium to large arteries and veins throughout the body can be affected, with veins more commonly involved than arteries. Sometimes, both arteries and veins are affected simultaneously. Large vessel involvement is more prevalent in males, occurs earlier than neurological manifestations, and affects younger individuals (average age 28.36 years).

The incidence of venous damage generally ranges from 10% to 46%. The primary pathology is thrombophlebitis and venous thrombosis, with occasional cases of esophageal varices. Superficial and deep veins may be affected simultaneously or separately. Among 30 cases of venous damage, conditions included superficial phlebitis, deep vein thrombosis of the lower limbs, and occlusion of the inferior or superior vena cava. The inferior vena cava is more frequently affected than the superior vena cava. Superficial vein involvement is often associated with severe fundus lesions, while inferior vena cava involvement may lead to leg ulcers or even progress to Budd-Chiari syndrome.

The incidence of arterial damage is generally 1.5% to 2.2%, predominantly affecting young and middle-aged males. It typically occurs when Behcet's disease is poorly controlled, appearing later in the disease course. The primary pathology is arteritis, leading to thrombosis, luminal stenosis, arterial dilation, and aneurysms. Among 84 cases of arterial damage, 24 involved aneurysms and 17 involved arterial occlusion. Aneurysms mainly occur in large arteries of the abdomen, lower limbs, chest, and neck, with fewer cases in the cerebral, coronary, pulmonary, and renal arteries. Occluded arteries are primarily the subclavian, pulmonary, common carotid, femoral, tibial, and radial arteries. Medium to large arteries throughout the body can be affected, either in a single location or sequentially in multiple arteries. Depending on the location, vessel size, type of damage, and progression, arterial occlusion and aneurysms can cause various clinical symptoms and signs. Of particular concern are pseudoaneurysms resulting from damage to the arterial media due to vasa vasorum involvement, which can rupture and lead to fatal hemorrhage. Superficial aneurysms are easily detectable, while deep ones are more challenging. Therefore, painless, pulsatile masses in Behcet's patients should be closely monitored. Additionally, after aneurysm resection, if the disease remains active, new aneurysms may develop elsewhere.

(7) Gastrointestinal Damage The incidence ranges from 8.4% to 27.5%. Symptoms include upper abdominal fullness and discomfort, belching, mid-to-lower abdominal distension, or intermittent colicky pain, hematochezia, and constipation more often than diarrhea. The primary lesions are ulcers, which can occur anywhere from the esophagus to the rectum, most commonly in the ileum and colon, particularly the distal ileum and ileocecal region. Ulcers present in three forms: typical ulcers, mainly in the ileocecal region, deep and penetrating; superficial aphthous ulcers, difficult to detect on barium studies but easier to identify via colonoscopy; and diffuse colonic ulcers resembling inflammatory bowel disease, ulcerative colitis, or Crohn's disease. Crohn's disease shares many similarities with Behcet's disease, and their relationship remains debated. Gastrointestinal and rectal endoscopy can reveal superficial ulcers in the lower esophagus, stomach, and rectum, with gastric ulcers less typical than peptic ulcers. Follow-up after 1–2 years may show ulcer resolution. Ileal ulcers may perforate, causing peritonitis, while ileocecal ulcers can mimic chronic appendicitis. Gastrointestinal involvement is more common in children than adults (50% vs. 29.3%).

(8) Nervous System Damage The incidence rate ranges from 2.8% to 10%, with males being more affected than females. The brain, midbrain, brainstem, cerebellum, spinal cord, meninges, cranial nerves, and spinal nerves can all be involved, with the brainstem, spinal cord, midbrain, and cerebrum being the most commonly affected sites. Brainstem involvement is the most severe. Primary lesions are mostly venulitis, capillaritis, or arteriolitis, while the most common secondary pathological changes are demyelination, followed by cerebral softening and perivascular inflammatory cell infiltration. Clinical manifestations vary depending on the location and pathological basis, such as encephalitis, meningoencephalitis, encephalomyelitis, or cranial nerve involvement (most commonly the abducens and facial nerves), as well as polyneuritis. A few cases present with symptoms of increased intracranial pressure and mental disorders, resulting in diverse clinical symptoms and signs that lack typical presentations. Nervous system involvement generally occurs later, and cases where the central nervous system is the initial manifestation of the disease are extremely rare. The progression of neurological conditions also follows a chronic course of recurrent episodes and remissions. The prognosis depends on the site of damage and timely intervention. The mortality rate in Chinese cases is 11.6%, lower than the 26–47% reported abroad, which may be related to the lower incidence of brainstem involvement.

(9) Pulmonary Damage Accounts for about 5%, mostly occurring between the ages of 30 and 40, with a male-to-female ratio of 9:1. The lesions may primarily involve pulmonary blood vessels, coexist with stirred pulse or venous lesions in other parts of the body, or result from emboli detached from venous thrombi in other areas that affect the lungs. The affected vessels include capillaries, small vessels, and stirred pulse and veins of varying sizes. Manifestations include vasculitis, thrombosis, thromboembolism or infarction, nodule formation, stirred pulse aneurysms, and diffuse interstitial fibrosis, among others. Clinical presentations vary widely depending on the type, size, and location of the affected vessels, as well as the nature and stage of the lesions. Common manifestations include unilateral or bilateral diffuse pulmonary inflammation or patchy shadows, interstitial pneumonia, bronchitis or peribronchial fibrosis, and pleuritis or pleural effusion. Pulmonary stirred pulse lesions may lead to stirred pulse inflammation or thrombosis; rupture of single or multiple stirred pulse aneurysms can cause massive pulmonary hemorrhage, leading to rapid death. Pulmonary stirred pulse hypertension may result in left ventricular hypertrophy. Thrombophlebitis or venous thrombosis may occur after pulmonary venous lesions, often accompanied by superior vena cava occlusion or thrombosis in other areas such as the cerebral or renal veins. Emboli from endocarditis or venous thrombi in other regions may also cause multiple pulmonary infarctions. Pulmonary infarction or bronchial membrane ulcers can lead to expectoration of blood, making hemoptysis a common and severe symptom in pulmonary involvement. The occurrence of multiple stirred pulse aneurysms in the lungs of young males, accompanied by thrombosis in other areas and concurrent oral, skin, and ocular lesions, clinically and pathologically resembles Hughes-Stovin syndrome, suggesting that the latter may represent a severe form of Behcet's disease.

(10) Renal Damage Renal involvement is rare, with the main lesions being glomerulonephritis or focal proliferative glomerulonephritis, which may present as intermittent proteinuria or microscopic hematuria. Occasionally, progression to amyloid deposition and nephrotic syndrome may occur.

During the course of the disease, acute testicular swelling and pain may occur, and rarely, sensorineural deafness may develop. Behcet's disease patients also frequently experience tonsillitis and pharyngitis.

bubble_chart Auxiliary Examination

During the active phase of the disease, markers such as "ESR," mucoprotein, sialic acid, and α2globulin are often elevated. Some patients test positive for plasma ceruloplasmin, and a few cases show positive cryoglobulin.

Observation of the tongue tip microcirculation may reveal atrophy of the fungiform papillae, which has auxiliary diagnostic value. For affected organs, corresponding examinations can be performed, such as ECG, EEG, CSF analysis, CT, MRI, X-ray imaging, fiberoptic gastroscopy and colonoscopy, angiography, and Doppler ultrasound.

bubble_chart Diagnosis

The diagnosis of this disease is mainly based on the clinical manifestations of commonly occurring lesions: ① Acute or chronic onset in the oral cavity, skin, genitals, and eyes; ② Certain characteristics of the lesions in these areas; ③ A chronic, recurrent, and remitting course of sexually transmitted disease.

In acute-onset cases, lesions in various areas often appear simultaneously or successively, presenting relatively clearly, making diagnosis seemingly straightforward. However, if the characteristics of the lesions are overlooked, misdiagnosis with other diseases affecting these areas—particularly drug reactions—can easily occur. In chronic cases, since lesions in different areas often appear separately and may not be detected simultaneously, misdiagnosis as fistula disease is common. Therefore, only by carefully taking the patient’s history, understanding the various lesions and their characteristics in each area, and combining this with the long-term, recurrent, and remitting course of sexually transmitted disease, can misdiagnosis and erroneous diagnosis as fistula disease be minimized. There are also many cases involving only two affected areas, lacking lesions in 1–2 of the commonly affected sites, while other manifestations are identical to those with 3–4 affected areas. These cases may also feature neurological, gastrointestinal, or large vascular lesions as a characteristic manifestation. If HLA-B5(+) is detected concurrently, it further aids in the diagnosis of this disease. The presence of erythema nodosum-like lesions with halos and folliculitis-like lesions with halos strongly suggests the diagnosis of this disease.

bubble_chart Treatment Measures

Since the disease cause is not yet fully understood, and clinical manifestations are diverse, sometimes the effect of a drug on lesions in different areas may vary. Therefore, treatment methods are also diverse.

  1. Life should be regular, with a balance of work and rest. When symptoms are significant, appropriate rest is advisable.
  2. For significant inflammatory lesions in the eyes, major blood vessels, central nervous system, joint swelling, and digestive tract, as well as high fever, corticosteroids such as prednisone 30–40 mg/d should be administered early, divided into multiple doses.
  3. For cases of thrombophlebitis or venous thrombosis, oral enteric-coated aspirin 0.3 combined with dipyridamole 25 mg, three times daily, or indomethacin 25 mg, three times daily.
  4. For occlusive stirred pulse lesions, Salvia tablets or compound Salvia tablets can be taken orally, 3 tablets each time, three times daily. For stirred pulse tumor diseases, surgical treatment should be performed early.
  5. For fundus lesions or bleeding, in addition to corticosteroids, cyclosporine or CB1348 can be used, or oral vitamin E 100 mg, three times daily.
  6. For nodular erythema-like lesions on the lower limbs, Chinese medicinals with the principles of invigorating blood and resolving stasis, clearing heat and removing toxin can be used. For example, tuckahoe peel 20g, Red Peony Root 10g, peony bark 10g, Peach Kernel 10g, iron tree leaves 30g, Barbated Skullcup Herb 30g, Hedyotis 30g, golden root 30g, sargenrgloryvine stem 30g, fragrant millet sprout 10g.
  7. For Sweet's disease-like lesions, oral antelope horn powder or concentrated buffalo horn powder, or 10% potassium iodide solution can be used.
  8. For cases with evidence of subcutaneous node infection, oral isoniazid 0.1 and ethambutol 0.25, three times daily, for one year.
  9. For mild, chronic, recurrent oral cases, oral thalidomide, 200–300 mg/d, divided into multiple doses.
  10. For long-term, chronic, recurrent mild cases, Root Leaf or Flower of Common Threewingnut preparations, colchicine 0.5 mg twice daily, as well as interferon and azathioprine, can be used.
  11. For oral, skin, genital, and eye lesions, depending on the type, topical medications of different properties and dosage forms, such as corticosteroid ointments or solutions, should be applied.

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