title: Pediatric scleroderma: systemic or localized forms.
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.
authors.affiliation: Division of Rheumatology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, PA 15224, USA. Kathryn.Torok@chp.edu
authors.forename: Kathryn S
authors.initials: KS
authors.lastname: Torok
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D003879:Dermatologic Agents / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011379:Prognosis; D012594:Scleroderma, Localized / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D016896:Treatment Outcome; D014467:Ultraviolet Therapy
chemical_list: D000893:Anti-Inflammatory Agents; D003879:Dermatologic Agents; D007166:Immunosuppressive Agents