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SCLERODERMA

SCLERODERMA. DEFINATION :- CHRONIC MULTISYSTEM DISORDER OF UNKNOWN ETIOLOGY CHARECTERISED CLINICALLY BY THICKENING OF SKIN CAUSED BY ACCUMULATION OF CONNECTIVE TISSUE AND BY STRUCTURAL & FUNCTIONAL ABNORMALITIES OF VICERAL ORGANS-GIT,LUNGS,HEART,& KIDNY. CLASSIFICATION OF SCLERODERMA.

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SCLERODERMA

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  1. SCLERODERMA • DEFINATION :- • CHRONIC MULTISYSTEM DISORDER OF UNKNOWN ETIOLOGY CHARECTERISED CLINICALLY BY THICKENING OF SKIN CAUSED BY ACCUMULATION OF CONNECTIVE TISSUE AND BY STRUCTURAL & FUNCTIONAL ABNORMALITIES OF VICERAL ORGANS-GIT,LUNGS,HEART,& KIDNY

  2. CLASSIFICATION OF SCLERODERMA • SYSTEMIC SCLEROSIS 1 .LIMITED CUTANEOUS DISEASE 2.DIFFUSE CUTANEOUS DISEASE 3.SINE SCLERODERMA 4.OVERLAPE SYNDROME • LOCALIZED SCLERODERMA 1.MORPHEA 2.LINER SCLERODERMA 3.En COUP de sabre • CHEMICALLY INDUCED SCLERODERMA • TOXIC-OIL SYNROME • VINYL CHLORIDE • BLEOMYCINE • PENTAZOCINE • EPOXI & AROMATIC HYDROCARBONES

  3. PATHOGENESIS HOST GENETIC SUSCEPTIBILITY INFECTION ENVIRONMENTAL FACTORS MICROCHIMERISM IMMUNE T CELL ACTIVATION MACROPHAGE ACTIVATION AUTOANTIBODIES CYTOKINES VASCULAR ENDOTHELIAL CELL INJURY VASCULAR OCCLUSION TISSUE HYPOXIA FIBROBLAST ACTIVATION FIBROSIS

  4. CLINICAL PRESENTATION OF S S • RAYNAUD’S PHENOMENON 90 TO 100 % • SKIN THICKENING 98 TO 100 % • TELENGIECTASIA 40 TO 85 % • ARTHRALGIAS 40 TO 70 % • ESOPHAGEAL DYSMOTILITY ~ 80 % • PULMONARY FIBROSIS ~40 % • MYOPATHY ~50 % • RENAL CRISIS ~ 15 % • CARDIAC DISEASE ~ 10%

  5. SUBSET OF SYSTEMIC SCLEROSIS

  6. MANAGEMENT • D-PENICILLAMINE 750 TO 1000 mg/DAY • IMMUNOSUPPRESIVE • CYCLOPHOSPHMIDE • CORTICOSTEROIDS • CYCLOSPORINE • ANTITHROMBOCYTE AGENTS • VASODILATORS • CALCIUM CHANNEL BLOCKERS-NIFEEDIPINE • PENTOXIPHYLLINE • SKIN CARE • PHYOTHERAPY

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