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Localized scleroderma in childhood

Author
Francesco Zulian, MD
Section Editors
Thomas JA Lehman, MD
John S Axford, DSc, MD, FRCP, FRCPCH
Deputy Editor
Elizabeth TePas, MD, MS

INTRODUCTION

Although scleroderma is a spectrum of disorders that can occur at any stage of life, the clinical patterns of childhood scleroderma differ from that of adulthood.

The predominant form of childhood scleroderma is localized scleroderma (LSc), also sometimes called morphea, which principally involves the skin, subcutaneous fascia, muscle, and bone (picture 1) [1-3].

Juvenile systemic sclerosis (JSSc) is a chronic, multisystem connective tissue disorder characterized by sclerodermic skin changes and abnormalities of the visceral organs. LSc rarely progresses into a systemic form [4-6] and can sometimes merge or overlap with eosinophilic fasciitis. (See "Eosinophilic fasciitis".)

This topic will review the incidence, etiology, diagnosis, and management of LSc in children. JSSc and the pathogenesis of SSc are discussed in detail elsewhere. (See "Juvenile systemic sclerosis (scleroderma)" and "Pathogenesis of systemic sclerosis (scleroderma)".)

DEFINITION

LSc has been differentiated from systemic sclerosis (SSc) based upon the clinical findings of almost exclusive cutaneous involvement (typically with some involvement of the underlying musculature) with absence of internal organ involvement. This distinction may not be as clear as previously thought as a significant minority of patients who present initially as LSc develop extracutaneous findings during the course of their disease. (See 'Extracutaneous manifestations' below.)

                    

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Literature review current through: Nov 2016. | This topic last updated: Wed Jun 29 00:00:00 GMT+00:00 2016.
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