Scleroderma

• Caused by fibrosis of the skin and superficial striated muscles
• Skin appears “tight”
• There are two main categories of scleroderma: Localized and Systemic
• Systemic Scleroderma is very rare in childhood, but is associated with pulmonary fibrosis and CREST phenomenon

Localized Scleroderma

• Two types: Morphea & Linear Scleroderma
• Morphea
  • plaques or drops of nontender, hard, & thickened skin with red/ violaceousborders that then becomes atrophied
  • Get both hypo and hyperpigmentation, creating a “salt and pepper” appearance
  • Can last years
• Linear Scleroderma
  • Bands of collagen;uni-or bilateral
  • Non painful, but can cause contractures and growth arrest if over a joint
  • Softens as patient enters adolescence
  • Looks like severe scarring, and can involve an entire half of the body, including muscle and bones, usually on legs
  • Tx: MTX

CHLA Board Review 2005