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Pediatr Clin North Am. 2012 Apr;59(2):381-405. doi: 10.1016/j.pcl.2012.03.011. Epub 2012 Apr 6.

Pediatric scleroderma: systemic or localized forms.

Author information

1
Division of Rheumatology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, PA 15224, USA. Kathryn.Torok@chp.edu

Abstract

Pediatric scleroderma includes 2 major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, with up to a quarter of patients showing extracutaneous disease manifestations such as arthritis and uveitis. Vascular, cutaneous, gastrointestinal, pulmonary, and musculoskeletal involvement are most commonly seen in children with SSc. Treatment of both forms targets the active inflammatory stage and halts disease progression; however, progress needs to be made toward the development of more effective antifibrotic therapy to help reverse disease damage.

PMID:
22560576
PMCID:
PMC3459339
DOI:
10.1016/j.pcl.2012.03.011
[Indexed for MEDLINE]
Free PMC Article

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