Thursday, February 28, 2008
What are the systemic scleroderma clinical manifestations
Systemic scleroderma - like onset occult. Renault started a common phenomenon, weakness, swelling of hands, such as arthritis or joint pain. With the slow development of illness, Typical damage to the skin and internal organs. Renault phenomenon about 70% of patients the first symptom of Raynaud's phenomenon may precede skin damage 1-2 years, or with skin symptoms simultaneously. The so-called Raynaud's phenomenon, that is, patients in the face of cold or mental tension, that (toe) terminal into a white, and then into a purple, the final red color change. With partial numbness; pain, soon after the general met warming mitigation for various periods of time, usually a few minutes to 10 minutes continued -. Renault phenomenon regular attack, a partial nutrition can change, such as that (toe) Tsim skin necrosis, and in proper scar. Nail ulcer may occur due to the depression caused by scar. Seriously, there will be the means, the end of the cold toes, bruised and pulp flattening, gangrene, that the distal bone resorption, dissolution, or change short cut means. Skin lesions of the skin lesions of systemic scleroderma vary greatly degree; most skin damage from the distal extremities or facial, symmetric distribution, gradually to the proximal and systemic progress can be involved serious systemic. Typical skin lesions generally have to go through three different stages: (1) diffuse edema of the skin mild swelling, a non - SAG, striae disappeared, smooth surface tension, or a pale yellow, skin temperature dropped, and consciously itching. After a few weeks or a few months later, the skin gradually enter the hardening phase. (2) hardening of the skin swelling subsided, gradually, acting like a wax-like surface gloss, smooth, a tawny, and subcutaneous tissue adhesion close, feeling numb or even disappear. (3) if the atrophy of parchment-like skin thinning, and even subcutaneous tissue, muscle atrophy occurred, hardening to keep the periosteum, a plank of atherosclerosis, high incidence of ulcers and gangrene. Violations of facial lesions, a mask-like: loss of facial expression, eyelid activities restricted; nasal tip small ear thinning, trismus, your lips thinning, I fissile small, the skin around the mouth contraction. Damage to the and there will be the shedding hair, reduce sweating, the lack of sebum. A wax intestinal smooth finger-like. That, toenail involvement, friable, shedding. The finger joints and stubborn ulcer-prone or avascular necrosis. Muscle articular lesions morning stiffness and joint swelling, pain common. Joint weeks tendon, fascia, skin fibrosis changed, the size of most of the patients in joint pain. Arthritis can be limited to single-joint or several joints, arthritis can be more to that, wrist and ankle common, all of which will ultimately involved joints. 'Arch that is characteristic signs. Early articular synovial inflammatory cell infiltration, surface fibrin synovial coverage dissipated late inflammation, synovial obvious fibrosis, or replaced by dense connective tissue. Joint activities of the leather-like friction sound. Smaller joint cavity, a flexion deformity. Buckling joint Department of chronic skin ulcers. Systemic scleroderma, there will be the muscle lesions, there are different degrees of muscle-ache, muscle atrophy and decreased muscle strength, such as increased muscle enzymes. Digestive oral mucosal lesions can occur sclerosis, atrophy, tongue activities can contracture and hardening of the restricted zone to be extended extraoral tongue. Periodontal gap widened, gingival marginal retreat, shedding teeth significantly. Esophageal dysfunction, expressed as dysphagia, swallowing, or substernal pain bulging nausea flu. If there esophageal inflammation, there will be the sternum after burning flu. Late to the disease, a result of esophageal stenosis, swallowing more difficult, and often accompanied with vomiting. Gastric rarely involved, but often intestinal involvement, there are abdominal distention, abdominal pain, diarrhea occurred alternatively. A small number of patients, there will be or obstruction, such as acute abdomen performance. Lung lesions of systemic scleroderma lung disease pulmonary fibrosis is the main performance for the conduct of activities after shortness of breath, activities tolerance is limited. Measurement of lung function can be found in gas diffusion dysfunction. Lung X-ray showed increased fiber texture to the two most obvious under the lung field, can be associated with cystic heavier shadow of a honeycomb or change. With the progress of the disease, there may be the pulmonary hypertension, eventually led to cor pulmonale. A small number of patients, there will be pleurisy, but pleural effusion rare. A heart attack myocarditis, pericarditis or endocarditis may have occurred, expressed as shortness of breath, chest Chuang, palpitation, ankle edema, angina, arrhythmias and syncope, can be caused by severe left ventricular failure or whole-hearted. Kidney damage kidney disease can be divided into acute and chronic two. Rapidly progressive renal damage often sudden onset, to the rapid development of malignant hypertension and renal insufficiency. This "systemic scleroderma renal crisis" usually occurs in winter, poor prognosis. Chronic renal damage in the pathogenesis more after three to five years after the start of intermittent proteinuria, and gradually developed into persistent proteinuria, microscopic hematuria, hypertension and azotemia. 'Nervous system lesions' carpal tunnel syndrome can occur, trigeminal neuralgia, facial paralysis, gastrointestinal autonomic nerve dysfunction, peripheral sensory disorders. CREST syndrome discretion systemic scleroderma is a special type, manifested as five characteristics of the joint, or skin calcinosis (C), Raynaud's phenomenon (R), esophageal reduce tension (E), means, toe skin hardening Youlu) and telangiectasia (T). Such patients often appeared in the blood of anti-centromere antibody positive. Typical rhyme with systemic scleroderma, visceral damage light of this syndrome, the slow progress saddle, the better prognosis.
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