The hallmark of the heterogeneous but related group of disorders called scleroderma is an area of hard, tight, inelastic (hide-bound) skin and subcutaneous tissue (Fig. 8.1).1,2 Fewer than 200 affected children have been reported on in the more than 130 years since the disorder was first described. The paucity of case reports does not reflect great rarity; one pediatric rheumatology clinic alone has reported 48 cases.3 It does reflect physician frustration: this disorder has been difficult to categorize; pathogenesis has defied understanding; treatment has been unsatisfactory.


Systemic Sclerosis Progressive Systemic Sclerosis Porphyria Cutanea Tarda Scleroderma Renal Crisis Localize Scleroderma 
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Copyright information

© Springer Science+Business Media New York 1993

Authors and Affiliations

  • Jerry C. Jacobs
    • 1
  1. 1.Section of Pediatric Rheumatology and the Regional Arthritis Program (RAP-4-Kids)Columbia-Presbyterian Medical CenterNew YorkUSA

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