Presentations and treatment of childhood scleroderma: localized scleroderma, eosinophilic fasciitis, systemic sclerosis, and graft-versus-host disease

Clin Pediatr (Phila). 2011 Jul;50(7):604-14. doi: 10.1177/0009922810396546. Epub 2011 Apr 27.

Abstract

Juvenile scleroderma is a rare connective tissue disease that involves the skin and subcutaneous tissue. Among all presentations of juvenile scleroderma, localized scleroderma (JLSc) is the most frequent, followed by systemic disease (JSSc) and eosinophilic fasciitis (EF). In posttransplantation chronic graft-versus-host disease (GvHD), scleroderma-like skin involvement can occur. Systemic forms of juvenile scleroderma and GvHD can affect the internal organs, such as the lungs, the gastrointestinal tract, the heart, and kidneys and cause disability and severe, sometimes lethal, complications. Here, the authors give an overview of different presentations of juvenile scleroderma. They report their experience with the different forms and presentations of scleroderma, diagnostic workups, treatment, and outcome of all forms of childhood scleroderma in the context of the existing literature.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Disease Progression
  • Eosinophilia / diagnosis*
  • Eosinophilia / drug therapy
  • Fasciitis / diagnosis*
  • Fasciitis / drug therapy
  • Female
  • Graft vs Host Disease / diagnosis*
  • Graft vs Host Disease / drug therapy
  • Humans
  • Infant
  • Male
  • Prognosis
  • Retrospective Studies
  • Scleroderma, Localized / diagnosis*
  • Scleroderma, Localized / drug therapy
  • Scleroderma, Systemic / diagnosis*
  • Scleroderma, Systemic / drug therapy