Vascular Manifestations of Systemic Sclerosis

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  • JINNIN Masatoshi
    Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University

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  • 全身性強皮症の血管病変
  • ゼンシンセイ キョウ ヒショウ ノ ケッカン ビョウヘン

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Abstract

<p>　Various manifestations are seen in the skin and internal organs of systemic sclerosis patients. The pathogeneses of these manifestations are considered to involve fibrosis, autoimmune dysfunction, and/or vasculopathy.</p><p>　Almost all patients have skin symptoms, which are classified into two groups: fibrotic lesions and vascular lesions. The fibrotic lesions include skin fibrosis, short sublingual frenulum, microstomia, mask-like face, and contracture of the phalanges, while Raynaud's phenomenon, skin ulcers, nail fold bleeding, pitting scars, telangiectasias, and pulmonary hypertension are well-known vascular lesions. These symptoms are present throughout the course of the disease and can severely affect patients' quality of life. However, their causative mechanisms are still to be clarified.</p><p>　Thus, this review aims to describe our current understanding of the clinical features of the vascular manifestations of systemic sclerosis, and discusses their pathogenic mechanisms and treatment.</p>

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