27 - Juvenile Systemic Sclerosis

Abstract

The scleroderma disorders in children encompass both systemic sclerosis (SSc) and localized scleroderma (LS), also termed morphea. Juvenile systemic sclerosis (JSSc) is much less common than juvenile localized scleroderma (JLS) and comprises less than 10% of all SSc cases. Both share the general histopathologic features of an initial inflammatory state, followed by collagen deposition and atrophy of the skin and underlying connective tissue. Clinically, areas of “hard skin,” which is the translation of the word scleroderma, result in uniting the two entities. Skin biopsies of LS and SSc patients are typically indistinguishable, and they may share some common pathophysiology; however, their clinical manifestations are notably different with unique morbidities and outcomes. LS is typically confined to the skin and underlying tissue, with some patients having extracutaneous disease manifestations relating to deeper tissue involvement, such as joint contractures and myositis, but rarely internal organ manifestations (see Chapter 28). In contrast, SSc affects the skin, vasculature, and internal organs of almost all patients, typically in a multiorgan fashion, with 38% children with SSc in a recent North American cohort study having four or more organ systems involved. The most commonly affected organ systems in JSSc are vascular (Raynaud phenomenon and digital ulceration), cutaneous (skin thickening), gastrointestinal, pulmonary, and musculoskeletal. The remainder of this chapter will focus on JSSc, for more information about juvenile localized scleroderma (morphea), please see Chapter 28.