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Juvenile systemic sclerosis (scleroderma)

Author
Francesco Zulian, MD
Section Editor
Thomas JA Lehman, MD
Deputy Editor
Elizabeth TePas, MD, MS

INTRODUCTION

Scleroderma is a spectrum of disorders that can occur at any stage of life, although the clinical patterns of childhood sclerosis differ from that of adulthood [1].

The predominant form of childhood sclerosis is localized scleroderma. Juvenile systemic sclerosis (JSSc) is uncommon but has the most significant morbidity of the sclerotic disorders.

JSSc is a chronic, multisystem, connective tissue disorder characterized by symmetrical fibrous thickening and hardening of the skin combined with fibrous changes in internal organs, such as the esophagus, intestinal tract, heart, lungs, and kidneys. The extent of skin involvement and the accompanying pattern of internal organ involvement form the basis for the classification of systemic sclerosis in children [2].

This topic will review the clinical presentation, diagnosis, and management of JSSc. Localized scleroderma and the pathogenesis of systemic sclerosis are discussed in detail elsewhere. (See "Localized scleroderma in childhood" and "Pathogenesis of systemic sclerosis (scleroderma)".)

DEFINITION

The principal types of systemic sclerosis include the following subsets in adults [3] (see "Overview and classification of scleroderma disorders"):

                               

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Literature review current through: Nov 2016. | This topic last updated: Wed Nov 09 00:00:00 GMT+00:00 2016.
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