The role of the intercellular matrix in dermal calcinosis of the CRST syndrome: An electron-microscopic study

V. Brazzelli, C. Dell'Orbo, G. Borroni, A. Bollati, C. Montecucco, D. Cerimele, G. Rabbiosi

Research output: Contribution to journalArticle

Abstract

We performed an electron-microscopic study on calcium deposits in two patients with calcifying scleroderma (CRST syndrome). Calcium deposits were detected both intracellularly in the mitochondria of phagocytic cells and extracellularly. Needle-like crystals measuring up to 4,500 Å in length and ~60 Å in width were present in both the Von Kossa-positive regions and the apparently normal dermal areas around the calcification sites. Although the fibrillar matrix's ultrastructure was normal, hollow oxytalan fibrils were detected. Slightly electron-opaque, star-shaped material was observed among the fibrillar component of the matrix (matrix granules), which is the ultrastructural expression of some types of proteoglycans containing keratan sulphate and condroitin sulphate. These granules cannot be detected in normal dermis. The extrafibrillary calcium deposits on these mucopolysaccharide structures may represent an early event in the complex pathogenesis of calcification in the CRST syndrome.

Original languageEnglish
Pages (from-to)42-49
Number of pages8
JournalAmerican Journal of Dermatopathology
Volume14
Issue number1
Publication statusPublished - 1992

Keywords

  • Calcinosis
  • CRST
  • Matrix granules
  • Proteoglycans

ASJC Scopus subject areas

  • Dermatology
  • Pathology and Forensic Medicine

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