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Systemic Sclerosis Scleroderma (Visit Resource)
A multi-organ disease (chest, bone, GI, etc.) of unknown etiology, affecting F> M. Immune? Abnormal collagen metabolism? Vascular abnormality? Clinically, Skin thickening (edema ->fibrosis) Muscle weakness Raynaud's phenomenon -- paroxysmal occlusion of digital arteries. Can be seen as isolated disease of associated with other entities including SLE, RA, dermatomyositis, mixed connective tissue disorder (MCTD). Dysphagia In the hands, Atrophy of soft tissue in finger tips Soft tissue calcification. Acro-osteolysis but DDx is extensive. Scleroderma HPTH Frostbite Leprosy Psoriasis Epidermolysis bullosa Polyvinyl chloride Multicentric reticulohistiocytosis Erosive osteoarthritis Lesch-Nyhan Syndrome Progeria In joints, Extensive involvement of 1st MCP with erosion, Ca, subluxation is characteristic. Erosions DIP, PIP must be distinguished from: RA PA Erosive osteoarthritis Elsewhere, metastatic calcification is seen, but must be differentiated from: Hypervitaminosis D Renal Osteodystrophy Milk alkali syndrome Other hypercalcemic states Dystrophic calcification
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Added: Sun Dec 17 2006
