TY - CHAP M1 - Book, Section TI - Chapter 157. Scleroderma A1 - Moinzadeh, P. A1 - Denton, Christopher P. A1 - Krieg, T. A1 - Black, Carol M. A2 - Goldsmith, Lowell A. A2 - Katz, Stephen I. A2 - Gilchrest, Barbara A. A2 - Paller, Amy S. A2 - Leffell, David J. A2 - Wolff, Klaus Y1 - 2012 N1 - T2 - Fitzpatrick's Dermatology in General Medicine, 8e AB - |PrintSynopsis at a GlanceSystemic sclerosis (SSc) is a multisystemic disease, characterized by excessive fibrosis, inflammation, and vasculopathy.The pathogenesis of this autoimmune process remains unclear.Differential diagnosis of SSc includes severe forms of localized scleroderma as well as many other scleroderma-like conditions.Patients with SSc are classified into two major subtypes depending on the extent of skin sclerosis [diffuse systemic sclerosis (dSSc) and limited systemic sclerosis (lSSc)]. Patients with an overlap syndrome, are characterized by additional clinical features of other rheumatic diseases.Raynaud phenomenon (RP) and skin sclerosis are almost always present.SSc is defined by sclerotic/fibrotic alterations of the skin and internal organs (digestive tract, lung, kidney, and heart), which can lead to severe dysfunction of almost any visceral organ.The heterogeneity and clinical course of SSc requires the urgent need of interdisciplinary collaborations and regular, at least yearly, follow-up visits.Although the disease is still not curable, there have been substantial advances in therapy for organ-based complications of SSc. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/24 UR - accessmedicine.mhmedical.com/content.aspx?aid=56076922 ER -