Pediatric scleroderma: systemic or localized forms

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


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Child
  • Dermatologic Agents / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Prognosis
  • Scleroderma, Localized* / complications
  • Scleroderma, Localized* / diagnosis
  • Scleroderma, Localized* / mortality
  • Scleroderma, Localized* / therapy
  • Scleroderma, Systemic* / diagnosis
  • Scleroderma, Systemic* / mortality
  • Scleroderma, Systemic* / therapy
  • Treatment Outcome
  • Ultraviolet Therapy


  • Anti-Inflammatory Agents
  • Dermatologic Agents
  • Immunosuppressive Agents