Juvenile Scleroderma


Scleroderma that occurs in children. Scleroderma is a connective tissue disease that can affect the skin, blood vessels, the immune system and sometimes even the organs may be involved. The disorder may be localized or affect large areas of the body.


  • Thickened skin 
  • Tight skin 
  • Patches of thickened skin 
  • Bands of tight skin 
  • Bands of thickened skin 
  • Loss of skin elasticity 
  • Impaired lung function 
  • Impaired heart function 
  • Impaired kidney function 
  • Esophageal dysmotility 
  • Movement problems 
  • Growth problems 
  • Joint stiffness 
  • Joint pain 
  • Fatigue 
  • Skin wasting 
  • Impaired immune system


Diagnosis is by clinical suspicion, presence of autoantibodies (specifically anti-centromere and anti-scl70/anti-topoisomerase antibodies) and occasionally by biopsy. Of the antibodies, 90% have a detectable anti-nuclear antibody. Anti-centromere antibody is more common in the limited form (80-90%) than in the systemic form (10%), and anti-scl70 is more common in the diffuse form (30-40%) and in African-American patients (who are more susceptible to the systemic form).


Prognosis for Juvenile Scleroderma. Localized scleroderma, such as linear, morphea, en coup de sabre, do not reduce a person's life expectancy. For children with systemic sclerosis (scleroderma), such as diffuse or limited/CREST, the survival rate is better for those who have adult onset. ISN.