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Related Experiment Videos

Extracellular microfibrils are increased in localized and systemic scleroderma skin.

R Fleischmajer1, L Jacobs, E Schwartz

  • 1Department of Dermatology, Mount Sinai School of Medicine, New York.

Laboratory Investigation; a Journal of Technical Methods and Pathology
|June 1, 1991
PubMed
Summary

Scleroderma involves increased deposition of collagen and 10-nm microfibrils in the dermis. These microfibrils, crucial extracellular components, stain for fibrillin, indicating their role in fibrotic skin conditions.

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Area of Science:

  • Dermatology
  • Connective tissue biology
  • Extracellular matrix research

Background:

  • Extracellular microfibrils (10 nm thick, hollow core) are found in most organs, often with elastic fibrils.
  • Scleroderma is characterized by fibrosis, a process involving connective tissue remodeling.

Purpose of the Study:

  • To investigate the role and composition of microfibrils in the fibrotic dermis of patients with localized and systemic scleroderma.

Main Methods:

  • Histochemistry was used to examine dermal tissue from scleroderma patients.
  • Immunofluorescence and immunoelectron microscopy were employed with antibodies against fibrillin and elastin.

Main Results:

  • Numerous fine elastic fibrils were observed in fibrotic dermal areas.

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  • An increase in 10-nm microfibrils, interspersed with collagen, was found in the lower dermis.
  • These microfibrils were identified as fibrillin-positive and elastin-negative.

    • Conclusions:

    • Fibrosis in both localized and systemic scleroderma is associated with the deposition of collagen fibrils and fibrillin-containing microfibrils.
    • Microfibrils are key components in the fibrotic process of scleroderma.