Management of Systemic Sclerosis-Related Skin Disease: A Review of Existing and Experimental Therapeutic Approaches

Abstract

The skin is the most common organ system involved in patients with systemic sclerosis (SSc). Nearly all patients experience cutaneous symptoms, including sclerosis, Raynaud's phenomenon, digital ulcers, telangiectasias, and calcinosis. In addition to posing functional challenges, cutaneous symptoms are often a major cause of pain, psychological distress, and body image dissatisfaction. The present article reviews the main features of SSc-related cutaneous manifestations and highlights an evidence-based treatment approach for treating each manifestation. This article also describes novel treatment approaches and opportunities for further research in managing this important clinical dimension of SSc.

Keywords: Cyclophosphamide (CYC); Diffuse cutaneous systemic sclerosis (DcSSc); Digital ulcers (DU); Hematopoietic stem cell transplantation (HSCT); Limited cutaneous systemic sclerosis (LcSSc); Methotrexate (MTX); Modified Rodnan skin score (mRSS); Raynaud’s phenomenon (RP).