Challenges and complications in juvenile localized scleroderma: A practical approach

Abstract

Juvenile localized scleroderma is characterised by inflammation which drives fibrosis in skin and soft tissues. The more severe subtypes of localized scleroderma such as linear and craniofacial are more common in children. Additionally, extracutaneous involvement is seen in half of all children and is associated with poorer treatment outcomes and health-related quality of life. Evidence for the management of craniofacial and extracutaneous involvement is lacking and therefore poses a challenge to clinicians. This review aims to provide a practical approach to management of these most challenging features of juvenile localized scleroderma through case studies where we present the available evidence, current recommendations and considerations for management.

Keywords: Craniofacial scleroderma; En coup de sabre; Extracutaneous involvement; Juvenile localized scleroderma; Linear scleroderma; Morphea; Parry Romberg syndrome.