Localized and systemic scleroderma.
Scleroderma is a broad term encompassing both localized and systemic sclerosis. Localized scleroderma is a cutaneous limited fibrosis that manifests as plaque morphea, generalized morphea, linear scleroderma, and deep morphea. Systemic scleroderma (sclerosis) can manifest as either limited or diffuse disease. Limited systemic sclerosis is typically preceded by Raynaud's phenomenon, involves cutaneous sclerosis distal to the elbows, with gastrointestinal and pulmonary fibrosis, and anticentromere antibody positivity. Diffuse systemic scleroderma is characterized by simultaneous Raynaud's phenomenon, cutaneous skin involvement proximal to the elbow with gastrointestinal, pulmonary, renal and cardiac fibrosis, and positive serology for antitopoisomerase and anti-RNAP III antibodies. This article discusses the classification, epidemiology, pathogenesis, clinical manifestations, treatment, and prognosis of the scleroderma.
Hawk
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Department of Dermatology, University of Virginia School of Medicine, Charlottesville 22908-0718, USA.
English
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D000328:Adult; D016026:Bone Marrow Transplantation; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002648:Child; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods*; D008297:Male; D011701:PUVA Therapy; D017893:Photopheresis; D011379:Prognosis; D011453:Prostaglandins / Q000627:therapeutic use; D012065:Relaxin / Q000627:therapeutic use; D012594:Scleroderma, Localized / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D012867:Skin / Q000473:pathology*
D002121:Calcium Channel Blockers; D011453:Prostaglandins; D012065:Relaxin