Scleroderma

Scleroderma is called systemic sclerosis. Clinical restricted or diffuse fibrosis and thickening of the skin characterized and involved heart, lung, kidney, digestive tract and other internal organs of connective tissue disease. All ages can develop the disease, but for the 20-50 age peak of incidence. The incidence rate for men is about 3-4 times. Scleroderma is Chinese "Paper weakness", "muscle weakness" areas, the main cause is due to weak-yang qi body, the blood-shortage disease is low, outside Zhu Fenghan evil exposed skin condensation pigs Jimmy, SYNDROMES passageway, leading to loss of body fluid fabrics, the body Haoshang, muscular dystrophy pigs, Stagnation context, the appearance of skin like hard leather, atrophy, poroma occlusive barrier is sweating obstacles Ganmao shedding, and other symptoms. Paper Bi run each day, there visceral disease. [1] The main symptoms, classification 1) Localized scleroderma include hardware spot disease, herpes scleroderma, a bit from scleroderma. 2) systemic sclerosis scleroderma including acromegaly, diffuse scleroderma, CREST syndrome (including acromegaly sclerosis and telangiectasia, calcium plot along, Raynaud's phenomenon, Esophageal dysmotility symptoms, such as systemic sclerosis of a type, to good. ) 2, symptoms and signs : 1. Localized scleroderma (a) hard spot disease : multiple in lumbar back, followed by the limbs and facial and neck, the performance of the round, Oval or irregular-shaped patch of edema, was the beginning of pink or purple, for a few weeks or a few months gradually expanding sclerosis, or light yellow color into Ivory, local-Khan, loss of hair, a few years later into a white or olive drab atrophic scars. Skin biopsy with scleroderma change. (2) Herpes scleroderma : occurs in children and youth, more females than males, and lesions along the intercostal side limbs were zonal distribution, for single or a few of the diseases with the evolution of hard spot. (3) Some hard-spot disease : multiple in the neck, chest, shoulder and back, and they were about to bean-fifth the size of coins, were - clustering, linear, its evolution seems hard spot. 2. Acral scleroderma and diffuse scleroderma acromegaly scleroderma have Raynaud's phenomenon, the skin lesions from distal to proximal development, trunk, less visceral involvement, slow disease progression, the prognosis is good; Diffuse scleroderma lesions from the trunk to the distal expansion, fewer Raynaud's phenomenon, more visceral involvement. Severe illness, disease progress faster to worse after. (1) Raynaud's phenomenon : the majority of patients the first symptom, performance as the (toe) terminal case of cold or emotional volatility when there Blushing → → redden bruising three-phase change, the weather can be eased; (2) Skin : disease process can be divided into edema, hardening and shrinkage of three. [Diagnostic Criteria -- Localized scleroderma skin under typical change can be diagnosed. System sclerosis : American Rheumatism Association (ARA) 1998 standards : A major criteria : metacarpophalangeal joints of the proximal Scleroderma changes can affect the whole body, face, body and trunk. B 418 : ① fingers scleroderma : The skin changes limited to finger; ② finger a depressed scar and refers to cushion disappeared. ③ basal lung fibrosis. Any one major criterion or two secondary standard for diagnosing other useful for the diagnosis of the performance : Renault, Multiple arthritis or joint pain, esophageal dysmotility, skin pathology collagen fibers swelling and fibrosis, Immunization checkup ANA, anti-Scl-70 antibodies, and centromere antibody (ACA) positive. CREST syndrome, in which five specific symptoms of the three, or three more plus centromere antibody-positive diagnosis can be. [Cause -- the etiology, pathogenesis unknown, and may be associated with the following factors : a genetic : in scleroderma patients, certain HLA class II antigen expression increased significantly applies. 2, learning materials and drugs : such as PVC, organic solvent, silicon, silicon dioxide, epoxy resin, L tryptophan, bleomycin, pentazocine such as scleroderma and offal induced fibrosis. 3, immune disorders : the existence of the disease humoral and cellular immune abnormalities, in the serum can be found anti-Scl-70-specific autoantibodies. Note the occurrence of the disease and immune disorders are closely related. 4, connective tissue disorder : the disease is a characteristic change of excessive collagen production, skin collagen were significantly increased. 5, the role of cytokines : certain cytokines in the pathogenesis of the disease, such as transforming growth factor, epidermal growth factor, platelet-derived growth factor. 6, vascular abnormalities : Most scleroderma patients performance Raynaud's phenomenon, pathology, Small artery intimal thickening and microvessel lumen stenosis or occlusion. [Laboratory] can be expressed as poor, hematuria, proteinuria, urinary tube, increased erythrocyte sedimentation rate, serum albumin decreased, globulin increased. - Based inspection : ANA positive rate of "90%, mainly spot - and NOR-type, about 20% of antibody-positive anti-RNP, 50%-90%CREST patients about anti-centromere antibody (ACA) positive (labeled antibody) 20 to 40% system sclerosis patients SCL-70 serum antibody positive (labeled antibody) RF 30% of the cases, peripheral blood T cell population normal or slightly lower, with the increase of T helper cells, T suppressor cells reduced.