The cause of disease
Its etiology is not fully understood, and it is currently believed to be related to the following factors.
1. Genetic factors
The incidence of systemic lupus erythematosus tends to be familial aggregation, and 0.4% ~ 0.5% of the first or second degree relatives of SLE patients suffer from LE or other autoimmune diseases. The prevalence of SLE in monozygotic twins can be as high as 70% (24% ~ 69%), while that in fraternal twins is 2% ~ 9%. At present, there are more than 50 gene loci related to SLE, most of which are HLA ⅱ and ⅲ genes, such as DR2, DR3, DQA 1, DQB 1 and C4AQ of HLA ⅲ gene in HLA ⅱ D region.
2. Sex hormones
This disease is more common in women of childbearing age, and pregnancy can induce or aggravate SLE. But the evidence is still insufficient.
3. Environmental factors and other factors
Ultraviolet irradiation can stimulate or aggravate LE, which may be related to its damage to keratinocytes. The damage is due to the change of DNA or the release of "hidden antigen" or the expression of new antigen, which leads to the formation of immune complex. Drugs such as phenylhydrazine, procaine, methyldopa, isoniazid and penicillin can induce drug-induced lupus erythematosus. Some infections (such as streptococcus, EB virus, etc. ) can also induce or aggravate this disease.
clinical picture
Discoid lupus erythematosus: it mainly invades the skin and is the lightest type of lupus erythematosus. A few cases may have mild visceral damage, and a few cases may turn into systemic lupus erythematosus. At the initial stage of skin injury, there are one or several bright red spots, ranging in size from mung beans to soybeans. There are scales attached to the surface, and then they gradually expand into a round or irregular shape, and the edge pigment is obviously deepened, slightly higher than the center. The center is pale, atrophic and low-lying, and the whole lesion is discoid (hence the name discoid lupus erythematosus). The damage is mainly distributed in sun-exposed parts, such as face, helix and scalp, and a few can involve upper chest, back of hand, forearm, lips and oral mucosa. Most patients have no symptoms, but it is difficult to completely disappear. The new injuries can be gradually increased or unchanged for many years, and the injuries are symmetrically distributed, or they can be merged into blocks, and the injuries in the middle part of the face can be merged into butterflies. Discoid lesions worsen after sun exposure or fatigue. Damage to the scalp can lead to permanent hair loss. Old injuries occasionally develop into squamous cell carcinoma of the skin.
Subacute cutaneous lupus erythematosus is a special intermediate type, which is rare in clinic. There are two kinds of skin lesions, one is annular erythema, which is single or multiple scattered erythema, annular, semi-annular or multi-annular. Dark red edge, slightly swollen and flushed at the outer edge, pigmentation and telangiectasia left after central regression, which is easy to occur on face and trunk; The other is papular scale type, and the lesions are similar to psoriasis, which are erythema, papules and plaques. There are obvious scales on the surface, mainly distributed in the upper limbs and face of the trunk. Most cases of two kinds of skin lesions exist alone, and a few can exist at the same time. Skin lesions often recur, and most patients have visceral damage, but rarely serious. The main symptoms are joint pain, muscle pain and repeated low fever, and a few have nephritis and blood system changes.
Systemic lupus erythematosus is the most serious type of lupus erythematosus. Most patients have multiple system damage at the time of onset, and a few patients develop from other types of lupus erythematosus. Some patients are accompanied by other connective tissue diseases, such as scleroderma, dermatomyositis, Sjogren's syndrome, etc., forming various overlapping syndromes. The clinical manifestations of systemic lupus erythematosus are diverse and complicated, and most of them are severe, which may endanger the lives of patients due to lupus nephritis, lupus encephalopathy and the side effects of long-term large-scale medication.
Deep lupus erythematosus, also known as lupus panniculitis, is also the intermediate type of lupus erythematosus. Skin lesions are nodules or plaques, located in deep dermis or subcutaneous adipose tissue, with uncertain size and quantity, normal or reddish skin color, solid texture and no activity. Injuries can occur anywhere, the most common are cheeks, hips, arms, followed by calves and chest. After chronic treatment, it can last for several months to several years, leaving skin atrophy and depression after healing. Deep lupus erythematosus is unstable and can exist alone, and then it can be transformed into discoid lupus erythematosus, systemic lupus erythematosus or both.
Neonatal lupus erythematosus, characterized by skin erythema annulata and congenital heart block, is self-trapping, usually resolves itself within 4 ~ 6 months after birth, and heart diseases often persist.
The main manifestations of drug-induced lupus erythematosus are fever, joint pain, muscle pain, butterfly erythema on the face, oral ulcer and serositis. ANA, anti-histone antibody and anti-ss-DNA antibody can be positive. After stopping the drug, it will gradually improve, and those with serious illness can be given glucocorticoid appropriately.
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Laboratory examination includes routine examination of blood, urine and feces, immunological examination, pathological examination of skin lesions, etc.
diagnose
DLE is mainly based on the characteristics of rash and skin pathological examination. If conditions permit, immunofluorescence band test can be done to help diagnose the disease. The diagnosis of SLE is mainly based on the history, clinical manifestations and laboratory examination. At present, the diagnostic criteria of SLE revised by American Rheumatology Association 1982 are generally adopted. If four or more of the 1 1 criteria appear successively or simultaneously, the patient can be diagnosed.
Syndrome differentiation and treatment of TCM
(A) the principle of treatment
This disease I mostly belongs to the syndrome of deficiency in the origin and excess in the origin, so it is necessary to distinguish between deficiency in the origin and excess in the origin. For those who focus on evidence, we should further distinguish between qi, nutrition and blood, which organs are involved, and the priorities of diseases; If the essence of deficiency is dominant, it is necessary to further distinguish between qi deficiency, yang deficiency and yin deficiency, and the location of the disease. Treatment should be based on the primary and secondary deficiency, and the corresponding treatment methods should be established. Because this disease is mostly caused by yin deficiency of liver and kidney and invasion of heat toxin, nourishing yin to reduce fire and clearing away heat and toxic materials are commonly used clinical treatments for this disease.
(2) treatment based on syndrome differentiation
1. Yin deficiency with internal heat syndrome
Main symptoms: facial erythema, clear edge, slight swelling, bright red or dark red, covered with scales, sun exposure or increased fatigue, accompanied by flushing on cheeks, low fever, fatigue, insomnia, night sweats, red tongue, little coating and rapid pulse.
Treatment: nourishing yin and tonifying kidney, clearing heat and cooling blood.
Prescription: Ziyin Liang Xue Decoction. Commonly used drugs: Rhizoma Anemarrhenae 12g, Rhizoma Paridis 12g, Cortex Phellodendri 12g, Radix Rehmanniae 30g, Cortex Moutan 12g, Lotus Seed Festival 12g, Rhizoma Imperatae 30g, buffalo horn 15g, and Radix Scrophulariae. Decoct Alisma orientalis 12g, Lycium barbarum peel 12g, Dendrobium nobile 12g, Artemisia annua 12g, and Ophiopogon japonicus 12g in water. Oral Baizhi Pills and Huadu Pills.
2. Qi stagnation and blood stasis syndrome
Main symptoms: facial erythema is butterfly-symmetrical, red in color, with gray scales, which are not easy to fall off, accompanied by hypochondriac swelling and pain, dizziness and pain, abdominal distension and anorexia, irregular menstruation or dysmenorrhea, pale tongue or petechiae, thin yellow fur, and astringent or thin pulse.
Treatment: soothing the liver and regulating qi, promoting blood circulation and removing blood stasis.
Prescription: Huoxue Huayu Decoction. Commonly used drugs: Bupleurum chinense 12g, Angelica sinensis 15g, Rhizoma Cyperi 12g, Radix Salviae Miltiorrhizae 30g, Rhizoma Chuanxiong 12g, Kochia scoparia 12g, Fructus Cnidii 12g, Radix Paeoniae Alba/kloc-0. Carthamus tinctorius 65438 02g, Fructus Aurantii 65438 02g, Fructus Toosendan 65438 02g, Herba Menthae 65438±00g, and Radix Glycyrrhizae 6g, decocted in water. Oral Jiawei Pill and Huoxue Pill.
3. Syndrome of excessive heat toxin
Main symptoms: acute onset, persistent high fever, erythema or ecchymosis on the skin, including hematemesis, bloody stool, purple spots, muscle soreness, joint pain, irritability and insomnia, delirium, convulsion, red tongue, yellow fur or falling off, and rapid pulse.
Treatment: clearing away heat and cooling blood, detoxifying and removing spots.
Prescription: Jiedu Huaban Decoction. Commonly used drugs: buffalo horn 12g, Radix Rehmanniae 30g, Cortex Moutan 12g, Radix Paeoniae Rubra 12g, Flos Lonicerae 20g, Rhizoma Paridis 15g, Rhizoma Polygoni Cuspidati 15g, Rhizoma Imperatae 30g, and Nodus Nelumbinis 12g. Oral pearl pills and antelope powder.
4. Deficiency of both Qi and Yin
The main symptoms: persistent erythema, bright red color, persistent low fever after high fever, fever in hands and feet, dry mouth and throat, upset and sleepless, shortness of breath and laziness, dull complexion, joint pain, alopecia, thin and white tongue coating, and thready pulse.
Treatment: invigorating qi and nourishing yin, dredging channels and activating collaterals.
Prescription: Yiqi Tongluo Decoction. Commonly used drugs: Radix Pseudostellariae 20g, Radix Ophiopogonis 12g, Radix Astragali 30g, Rhizoma Polygonati Odorati 12g, Fructus Corni 12g, Rhizoma Anemarrhenae 12g, Fructus Schisandrae Chinensis 12g, and Radix Rehmanniae 15g. Shengmai Pill and Tongmai Pill are taken orally.
5。 syndrome of yang deficiency of heart and kidney
Main symptoms: erythema of skin lesions is not obvious or there is no erythema, with cold form and cold limbs, aching waist and knees, listlessness, swollen face and floating limbs, palpitation and shortness of breath, cold abdomen and loose stool, fat and tender tongue, pale tongue with teeth marks, white fur and deep and thin pulse.
Treatment: Warming yang and tonifying kidney, promoting blood circulation and dredging collaterals.
Prescription: Bushen Tongluo Decoction. Commonly used drugs: Radix Aconiti Lateralis Preparata 10g, Cortex Cinnamomi 6g, Radix Rehmanniae 20g, Cornus officinalis meat 15g, Rhizoma Dioscoreae 30g, Cortex Moutan 12g, Poria 12g, Alismatis Rhizoma 12g, Ginseng 6g, Radix Astragali 30g, Atractylodis Rhizoma 65438. Take Shenfu Pill and Zhuyu Pill orally. (Cao Yuancheng)