What Are the Different Types of Scleroderma?
The occurrence of Raynaud's phenomenon (that is, in dry and cold weather, the extremities of the limbs will turn pale, but will turn red when the blood vessels re-open) and the skin becomes hard and thick.
Scleroderma
- The pathogenesis of scleroderma is still unclear, but abnormal fibrosis and vascular wall stenosis caused by thickening of the blood vessel wall can be observed in the body.
Common symptoms of scleroderma
- The occurrence of Raynaud's phenomenon (that is, in dry and cold weather, the extremities of the limbs will turn pale, but will turn red when the blood vessels re-open) and the skin becomes hard and thick.
- Gastrointestinal tract: Includes difficulty swallowing, reflux of acid in the stomach, and decreased bowel movements.
- Lungs: Interstitial lung fibrosis and pulmonary hypertension are currently the most common causes of death in systemic sclerosis.
- Kidney: The most serious is renal hypertension crisis, which refers to sudden hypertension due to vascular disease, which causes rapid deterioration of renal function, vision
- Scleroderma
- Heart: A few can have arrhythmia, heart failure, pericarditis, and so on.
- Musculoskeletal: General joint pain and morning stiffness are typical symptoms.
- Malignant tumors: Patients with scleroderma are more likely to develop lung tumors than the average person.
Scleroderma in terms of treatment
- Skin: It is very important to keep warm in cold weather. Vasoconstrictors should be avoided. Drugs such as calcium blockers can be used to increase local blood flow. As for the proliferation and thickening of the skin, no drug has sufficient curative effect.
- Lungs: Pulmonary fibrosis is an irreversible change. Early treatment may increase hope for treatment. Cyclophosphamide treatment trials are still ongoing. Intravenous vasodilators can temporarily relieve pulmonary hypertension, and lung transplantation can be considered in severe patients.
- Renal crisis: A small number of patients require short or long-term dialysis treatment.
- Scleroderma is now called systemic sclerosis. It is clinically characterized by localized or diffuse skin thickening and fibrosis, and involves connective tissue disease of internal organs such as heart, lung, kidney, and digestive tract. Onset can occur at all ages, but the peak incidence is 20-50 years old. The incidence of women is about 3-4 times that of men.
- Scleroderma approach mental disorders
The main symptoms of scleroderma
Classification of scleroderma
- 1) Localized scleroderma includes scleroderma, zonal scleroderma, and spotted scleroderma.
- 2) Systemic sclerosis includes acral scleroderma, diffuse scleroderma, and CREST syndrome (including acral sclerosis and capillary dilatation, calcium accumulation, Raynaud's phenomenon, and abnormal symptoms of esophageal motility, which is systemic A type of hardening, the prognosis is better.)
Scleroderma symptoms and signs
- Limited scleroderma
- Sclerotinia scleroderma: most often in the waist and back, followed by limbs and face and neck, and appear as round, oval or irregular edema patches, which appear initially red or purple, after several weeks or months Gradually expand and harden, the color becomes pale yellow or ivory, local no sweat, hair loss, and after several years, it turns into white or light brown atrophic scar. Skin biopsy was consistent with scleroderma changes.
- Zerosclerosis: It is more common in children and young people. There are more women than men. The lesions are distributed along the intercostal and side limbs in a band-like manner. The lesions can be single or multiple.
- (3) Dotted hard spot disease: It occurs frequently in the neck, chest, shoulders, etc., and is about the size of mung beans to five cents.
- Metatarsal scleroderma and diffuse scleroderma
- Raynaud's phenomenon of acral scleroderma, skin lesions develop from distal to proximal, less involvement of trunk and viscera, slow progress of disease course, good prognosis; diffuse
- Raynaud's phenomenon: It is the first symptom of most patients, which manifests as whitening blue-purple reddish three-phase change when the (toe) end is cold or emotional fluctuations; it can be relieved after warming
- skin: the disease process can be divided into three stages of edema, sclerosis and atrophy.
Scleroderma diagnostic criteria
- Local scleroderma can be diagnosed based on typical skin changes.
- Systemic sclerosis: American Rheumatology Association (ARA) 1998 standard:
- A main criteria: crust changes at the proximal end of the metacarpophalangeal joints, which can affect the entire limb, face, body and drive dryness.
- B secondary criteria: finger scleroderma: the above skin changes are limited to fingers;
- Scarring scars on fingertips and disappearance of finger pads;
- Basal fibrosis of both lungs.
- 1 major criterion or 2 minor criteria can be diagnosed, other manifestations that help diagnosis: Raynaud's phenomenon, multiple arthritis or joint pain, abnormal esophageal peristalsis, dermatological collagen fiber swelling and fibrosis, immunological examination ANA Anti-Scl-70 antibody and centromere antibody (ACA) were positive.
- CREST syndrome, specifically 3 of the 5 symptoms, or 3 or more with a centromere antibody can be diagnosed.
Causes of Scleroderma
- The etiology and pathogenesis of the disease are unknown and may be related to the following factors:
- Genetics: In patients with scleroderma, the expression of certain HLA-II antigens is significantly higher than that of ordinary people.
- Chemicals and drugs: such as polyvinyl chloride, organic solvents, silicon, silicon dioxide, epoxy resin, L-tryptophan, bleomycin, pentazocine, etc. can induce fibrosis of crusts and viscera.
- Immune abnormalities: The disease has humoral and cellular immune abnormalities. Specific anti-Scl-70 autoantibodies can be found in the patient's serum. This shows that the occurrence of this disease is closely related to immune disorders.
- Connective tissue metabolism abnormality: The characteristic change of this disease is excessive collagen production, and the collagen content in the skin is significantly increased.
- Vascular abnormalities: Most patients with scleroderma show Raynaud's phenomenon. Pathology shows that the intima of small arteries and microvessels is thickened, and the lumen is narrowed or occluded.
Scleroderma laboratory test
- Can be manifested as deprivation, hematuria, proteinuria, cast urine, increased erythrocyte sedimentation, decreased serum albumin, and increased globulin.
- Exemption from testing: ANA positive rate> 90%, mainly spotted and nucleoli, about 20% anti-RNP antibody positive, about 50% -90% of CREST patients with anti-centromere antibody (ACA) positive, (labeled antibody ), 20% -40% of patients with systemic sclerosis are positive for SCL-70 antibody (labeled antibody), 30% of cases have RF sex, the total number of peripheral blood T cells is normal or slightly lower, of which T helper cells increase, T suppressor cells cut back.
- [Clinical efficacy] Treatment of 560 cases of this disease, including localized 340 cases and systemic 220 cases. Results 541 cases were cured (the skin color and elasticity returned to normal), and 19 cases were effective (the skin became soft and the elasticity recovered, but there was still slight depression and atrophy), with a total effective rate of 100%.
TCM characteristic treatment of scleroderma
Overview of Scleroderma
- Scleroderma is an autoimmune disease of unknown origin, with pale or reddish skin edema, followed by dry, smooth, thickened, hardened, thinned, and shed hair, prone to stubbornness at the fingertips and joints Ulcers are the main clinical features. The disease is localized and systemic. The former is limited to skin and muscle damage, while the latter also affects the internal organs. Scleroderma is more common at the age of 20-50 years, and more women than men
- Scleroderma Treatment
Etiology and pathology of scleroderma
- The cause of scleroderma is divided into internal cause and external cause. The internal cause is insufficient due to innate endowment, and the external cause is wind, cold, damp heat, and other evils, which cause obstruction of the meridians and blood disorders.
- The pathological changes were evils such as wind chills or heat poisoning in the lung health, the lungs were lost, the camps were lost, the qi and blood were not working well, and the obstruction of the veins was obstructed, but the joint muscles were sore and the skin was hard. Qi is blood handsome, qi stagnation is blood stagnation; yang is the main temperature transport, yang deficiency is cold coagulation, which can cause blood stasis and blood loss, but the skin is hard as wax. The evil love is long, the righteousness is greatly lost, the essence and blood is lost, and the internal organs are affected by the surface and the inside.
Essentials for scleroderma diagnosis
- In the early stage, unexplained edema and symmetrical diffuse sclerosis occurred in the back of the hand and upper eyelid, late skin sclerosis and finger flexion contracture.
- Extremities often have Raynaud's phenomenon, as well as intractable ulcers and scars, multiple joint pain and arthritis, or difficulty swallowing and breathing.
- Skin biopsy of the forearm extension of the forearm showed swelling or fibrosis of collagen fibers and similar changes in the vessel wall.
Scleroderma syndrome differentiation
- Symptoms of wind cold and collaterals: aversion to cold, slight heat, hard skin, sore muscles and joints, or white cough and phlegm. The tongue is reddish, the fur is thin and white, and the veins are tight.
- Syndrome analysis: the wind and cold strike the table, the camp guards are out of sync, and the cold is feverish;
- Lung loss is measured by cough and white phlegm; evil stagnation of the collaterals and qi and blood are unfavorable, then the skin is hard and the muscles and joints are sore; the tongue is red, the moss is thin and white, and the veins are tight. Sign.
- Symptoms of fever and stagnation: fever, cough, shortness of breath, pain in muscles and joints, numbness, wet or dry necrosis of the toes. Tongue red, yellow dry or less bitter, pulse count.
- Syndrome analysis: fever invasion, enter lung health first, lung loss is declared, fever, cough, shortness of breath; evil stagnation, heat and internal force, qi and blood operation is unfavorable, muscle joint pain, numbness; evil enter camp Blood, heat and internal force, and stasis and heat rot are measured by wet or dry necrosis at the toe end; red tongue, yellow or dry fur or less fur, and pulse count are all signs of heat and internal depression, and enter the camp for injury.
- Symptoms of qi qi stagnation and blood stasis: dark complexion, thin skin, hard skin, and difficulty swallowing. The tongue is dull, the fur is thin, and the veins are thin.
- Syndrome analysis: Qi stagnation and blood qi stagnation, blood stasis, muscle stagnation, loss of Confucianism, you will see dark complexion, thin skin, hard skin; active qi, qi stagnation, difficulty in swallowing mouth; Darkness, thin moss, and thin pulses are signs of qi stagnation and blood stasis.
- Symptoms of cold coagulation and stasis: chills, cold limbs, joint pain, tight muscles and swelling, hard wood like wax. The tongue is pale and dark, the moss is gray and smooth, and the veins are thin.
- Syndrome analysis: weak yang, cold condensing meridians, blood stasis, chills, cold limbs, joint pain, and muscle swollenness; blood stasis caused deficiency, skin loss of glory, the operation is hard like wax; tongue is fat and dark , The moss is gray and slippery, and the pulse is thin, all of which are signs of yang deficiency and cold coagulation and stasis.
- Symptoms of Zhengxue and Xuanlian: thin limbs fatigue, palpitation, shortness of breath, dizziness, whiteness, muscle pain, thin skin like hard paper, withered skin, and hair loss. The tongue is pale, the fur is thin, and the veins are weak.
Analysis of Scleroderma Syndrome
- The evil love is long, the righteousness is greatly deficient, the two internal organs are deprived of vitality. Heart main blood, lung main gas, cardiopulmonary insufficiency are pale, palpitation, shortness of breath; spleen main muscles, main limbs, kidney main essence, raw marrow filling brain, spleen and kidney insufficiency, thin limbs tired, dizziness, hair loss; due to deficiency And stasis, skin and muscle loss of qi and blood for a long time, muscle pain, skin thin as hard paper, withered skin; pale tongue, thin moss, weak pulse, all are a sign of righteousness, deficiency of qi and blood.
Scleroderma typing
- Lung Weibi resistance rule: Xuanfei scattered cold, Huoxue Tongluo.
- Recipe: Jingfang Baidu Powder added and subtracted.
- Nepeta 9 grams of windproof 9 grams of Bupleurum 9 grams of cricket live 9 grams of Angelica 12 grams of Chuanxiong 12 grams of Qianhu 9 grams of bellflower 6 grams of licorice 6 grams of licorice 9 grams of cinnamon sticks to express sweating; those with obvious joint pain, add 15 grams of weilingxian, 15 grams of self-protection, 15 grams of osteospermum, 9 grams of hematoxylin to strengthen the cold and dispelling wind, Tongluo blood circulation and pain relief .
- heat toxin and stagnation rule: Qingrejiedu, Liangying Tongluo.
- Recipe: Yinqiao Sanhe Qingying Decoction.
- Honeysuckle 15g Forsythia 12g Chinese bellflower 6g Burdock 12g Raw 15g Black ginseng 12g Red sedge 15g Danpi 12g Purple grass 15g Ground grass 15g Honeysuckle
- The rule of qi qi stagnation and blood stasis: regulating qi and promoting blood circulation, removing blood stasis and clearing collaterals.
- Recipe: Body pain Zhuyu Decoction.
- Chuanxiong 9 grams peach kernel 9 grams safflower 9 grams angelica 9 grams Wulingzhi 9 grams (pack) incense with 9 grams achyranthes 9 grams Qin Zhi 9 grams live 9 grams dilong 12 grams with symptoms: skin thinner, add chicken 15 grams of blood rattan and 30 grams of astragalus to help nourish qi and nourish blood; those with blood stasis and fever, add 9 grams of rhubarb and 9 grams of tannin to eliminate stasis and clear heat.
- Zhi Han coagulation and stasis treatment: Wenyang Sanhan, Tongluo Huayu.
- Recipe: Yanghe Decoction.
- Angelica sinensis 9 grams ephedra 9 grams cooked 15 grams antler gum 9 grams (simmered) white mustard 9 grams cannon ginger 6 grams cinnamon 3 grams (back) 9 grams of safflower For patients, add 15 g of tablets and 12 g of Eucommia to warm the kidneys and strengthen the waist; for those who have diarrhea and greasy skin, go to the maturity and add 10 g of Atractylodes root and 30 g of Coix seed to strengthen the spleen and dampen; 9 grams of vinegar vinegar, 15 grams of kelp, and seaweed each to soften stasis.
- Zhengxue Xielian rule: Yiqi Yangxue, Huoxue Tongluo.
- Recipe: Sijunzi Decoction and Taohong Siwu Decoction.
- Astragalus 30 grams Codonopsis 15 grams Atractylodes 12 grams Poria 15 grams Angelica 10 grams Chuanxiong 10 grams peach kernel 9 grams safflower 9 grams chicken blood vine 15 grams dilong 9 grams add or subtract with symptoms; those who have obvious muscle and joint pain, add Qin Zhi 15 grams, 15 grams of Chenglingxian, 6 grams of frankincense, and myrrh, to relieve wind and relieve pain.
Chinese medicine for scleroderma
- Quanlu Wan 6 grams each time, 3 times a day.
- Xiao Huoluo Dan; 6 grams each time, 3 times a day.
- Shiquan Dabu Wan 1 capsule each time, 3 times a day.
Scleroderma Simple Formula
- 30 grams of astragalus, 9 grams of angelica, 9 grams of cinnamon sticks, 30 grams of heliotrope, 12 grams of antlers, decoction, 1 dose daily. For qi and blood deficiency, Yang deficiency blood stasis type dermatosis.
Other treatments for scleroderma
- External treatment method: Guizhi, Chuanxiong, Sumu, Safflower, Asarum, Artemisia spp. 20-45 grams each, decoct, soak or fumigate the affected limbs, hands and feet, 20-40 minutes each time (maintain the temperature of the medicine). 1 to 2 times a day, 1 course in January.
- Acupuncture: Acupoints are divided into four groups. Dazhui and Shenshu; Mingmen and Pishu; Qihai and Xuehai; Geshu and Feishu. The above four groups take turns acupuncture, which is mainly supplementing, and can be given moxibustion. 3 times a week.
- Acupoint injection method: Angelica injection 2 4ml, choose the above group for local injection, once a day or every other day, 10 days is a course of treatment.
- Manipulation method: Pressing, pressing, rubbing, pushing, point, dialing and other methods to massage at the acupoints related to the limbs. Once a day, 30 times is a course of treatment. The technique has a certain intensity, and the patient's tolerance is the degree.
Scleroderma precautions
- Scleroderma has no effective treatment at present. Long-term Chinese medicine treatment should be adhered to.
- Abortion and abortion can cause the disease to worsen, so pregnancy should be avoided.
- Pay attention to cold protection, especially to avoid cold water stimulation and maintain limb temperature.
- Actively remove infected lesions to prevent fatigue and severe mental stimulation.
- Typical scleroderma process
- Skin changes are a hallmark of scleroderma. However, the extent of the lesions is very different. Mild cases have only localized skin sclerosis and calcification, and severe cases may have widespread skin sclerosis and thickening. Typical skin lesions go through three stages of swelling, infiltration and atrophy in turn. The lesions are symmetrical. Most of the lesions gradually expand from the fingers to the proximal end, and the boundary between the diseased skin and normal skin is unclear.
Scleroderma swelling
- Symmetric, non-depressive, painless swelling, tense and thickened skin, wrinkles disappear, pale skin, fingers are salami-like, and develop proximally, and the back of the hand is swollen, and sometimes the forearm also has the same changes. There are also a few that develop from the trunk and gradually expand around them.
Scleroderma infiltration
- After a few weeks or months, the skin moves from the swollen phase to the infiltrated phase. At this time, the skin thickens, hardens, loses elasticity, and adheres to deep tissues. It cannot move, cannot be pinched, and is as bright as leather. The lesions can affect the fingers, back of hands, limbs, trunk and face. Facial features are characteristic mask-like changes, lack of expression, reduced wrinkles, and restricted eyelid movement; difficulty in opening the mouth. When opening the mouth, try to reduce the maximum distance between the upper and lower lips significantly than normal people (less than 3.5cm); A large number of radial wrinkles appeared around the lip, the nose became smaller and sharper, and the nose atrophy became softer. The face, neck, and lip mucosa appeared as a number of small capillaries. Occurs in fingers and can make finger flexion unfavorable. Occurs in the chest with a tight feel.
Scleroderma atrophy
- After another 5-10 years, skin damage enters the atrophic period. At this time, skin tightening becomes inconspicuous, and it even becomes thinner and softer to varying degrees, with a smooth appearance like kraft paper, and sometimes pigmentation or loss. The atrophy period is the earliest to appear on the latest affected skin, and hair and hair on the affected area may fall off. Due to the atrophy of the skin and loss of elasticity, the skin at the joints of hands is prone to ulcers after friction and collision. Calcification of subcutaneous soft tissue is a late complication of scleroderma. It is the result of local chronic inflammation. It occurs in vulnerable areas such as fingertips, elbows, and knees. Deeper small calcified nodules found.
- Common causes
- (1) Immune abnormal scleroderma is an autoimmune disease that often overlaps with other systemic or organ-specific autoimmune diseases. The most common are systemic lupus erythematosus and polymyositis, but also rheumatoid arthritis Sjogren's syndrome; organ-specific autoimmune diseases such as Hashimoto's thyroiditis and primary biliary cirrhosis. Most patients with this type of scleroderma have clinical characteristics and specific laboratory abnormalities associated with the disease, but they are not sufficient to diagnose the second disease. (2) Vascular scleroderma has a wide range of vascular lesions, including middle arteries, arterioles, arterioles, and capillaries, and occasionally involves aorta, making the skin, gastrointestinal tract, lung, heart, kidney, and finger-tip arteries all Because of the involvement, some people think that scleroderma is the result of a vascular endothelial disease, that is, the primary vascular damage is a significant intimal hyperplasia, and the cells are arranged concentrically in the middle, which is called endothelial cell mucoid changes. Vascular manifestations of scleroderma include early edema, Raynaud's phenomenon, capillary dilation, capillary abnormalities, and a wide range of multisystem vascular diseases. Fleischmajer et al. Used electron microscopy to find that the earliest changes in capillaries in patients with scleroderma were large fissures between endothelial cells. These fissures allowed extravasation of plasma into the extracellular matrix, which caused early edema before the characteristic fibrotic phase. . After the fissures occur, the cells swell and then break down, and the organelles are released into the lumen, resulting in capillary occlusion. This repeated destruction of endothelial cells causes the replication of substrates, each substrate layer corresponding to a remnant of endothelial cell regeneration. (C) The abnormality of collagen metabolism, both morphologically and biochemically, suggests that skin and visceral fibrosis in patients with scleroderma are the result of hyperproliferation of collagen tissue. Scleroderma is most clinically characterized by thickened and severely hardened skin. The stability of the skin structure depends almost entirely on fibrous collagen, and changes in the quality and quantity of collagen cause the symptoms and signs of scleroderma. In scleroderma, fat is replaced by collagen in the subcutaneous tissue and tissues surrounding the sweat glands. Fleischmajer pointed out that when examined under an optical microscope, the dermis showed dense connective tissue and collagen in a large bundle shape due to the narrowing of the interfascicular space, the hair body staining was normal, and the hyaline connective tissue hairy body staining was light The degree of coloration is composed of fine fibers, and the two can be identified.
Scleroderma Scleroderma Complications
- (1) joint damage;
- (2) damage to the smooth muscles of the gastrointestinal tract;
- (3) malnutrition;
- (D) formation of fibrous tissue in the myocardium which may cause permanent damage and / or functional degradation;
- (5) kidney damage and / or renal dysfunction;
- (6) The thyroid gland forms fibrous tissue.