Glycosaminoglycan alteration in the skin of children with classical Ehlers-Danlos syndrome

Acta Derm Venereol. 1985;65(6):489-94.

Abstract

Three children with classical Ehlers-Danlos syndrome were studied. Electron microscopic observation of the skin demonstrated a hyaluronic acid-dominant glycosaminoglycan structure in the intercellular matrix. Biochemical analysis showed a significant increase in hyaluronic acid content, as compared with the skin of sex- and age-matched children, including a non-affected brother of a patient. In addition, enhanced hyaluronic acid synthesis was demonstrated in vitro, using a patient's dermal fibroblasts. Analysis of the collagen showed no apparent abnormalities.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Child, Preschool
  • Collagen / metabolism
  • Dermatan Sulfate / biosynthesis
  • Ehlers-Danlos Syndrome / metabolism*
  • Ehlers-Danlos Syndrome / pathology
  • Fibroblasts / metabolism
  • Glycosaminoglycans / metabolism*
  • Histocytochemistry
  • Humans
  • Hyaluronic Acid / biosynthesis
  • Male
  • Microscopy, Electron
  • Skin / metabolism*
  • Skin / ultrastructure

Substances

  • Glycosaminoglycans
  • Dermatan Sulfate
  • Hyaluronic Acid
  • Collagen