Cutaneous Manifestations of Scleroderma

Abstract

Scleroderma is a rare, female predominant clinically heterogenous, autoimmune condition characterized by the presence of autoantibodies, vascular abnormalities, and fibrosis. Skin thickening as a result of fibrosis is a hallmark clinical finding, and the extent of this skin thickening further classifies clinical subsets of scleroderma. Organ systems affected may include pulmonary (interstitial lung disease, pulmonary arterial hypertension), renal (scleroderma renal crisis), gastrointestinal (dysmotility, reflux esophagitis, gastric antral vascular ectasia), cardiac (arrhythmias, cardiac fibrosis), and musculoskeletal (arthritis, contractures). Raynaud phenomenon and telangiectasis occur as a result of vascular involvement. Diagnostic workup requires a thorough history and physical examination, supplemented by autoantibody testing. No single therapeutic agent is indicated to treat all manifestations of scleroderma. Rather, system- or organ-based treatment is indicated. Not only are skin manifestations often the presenting feature of scleroderma, progressive skin changes carry prognostic implications, underscoring the value of dermatology insight for early diagnosis suspicion, referral to rheumatology, and timely management.