Cutaneous Manifestations of Scleroderma

Manushi Aggarwal, V. Sandhu
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Abstract

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.
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硬皮病的皮肤表现
硬皮病是一种罕见的、以女性为主的临床异质性自身免疫性疾病,其特征是存在自身抗体、血管异常和纤维化。由于纤维化导致的皮肤增厚是一个标志性的临床发现,这种皮肤增厚的程度进一步分类硬皮病的临床亚群。受影响的器官系统可能包括肺(肺间质性疾病、肺动脉高压)、肾(硬皮病肾危象)、胃肠(运动障碍、反流性食管炎、胃窦血管扩张)、心脏(心律失常、心脏纤维化)和肌肉骨骼(关节炎、挛缩)。雷诺现象和毛细血管扩张是血管受累的结果。诊断检查需要全面的病史和体格检查,并辅以自身抗体检测。没有单一的治疗药物可以治疗硬皮病的所有表现。相反,建议采用系统或器官为基础的治疗。不仅皮肤表现通常是硬皮病的表现特征,进行性皮肤变化还具有预后意义,强调了皮肤病学对早期诊断、怀疑、转诊到风湿病学和及时治疗的价值。
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来源期刊
CiteScore
0.30
自引率
25.00%
发文量
45
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