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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.
Abstract:
Extracellular microfibrils, about 10 nm thick with a hollow core have been found in most organs as free bundles or in association with elastic fibrils. Histochemistry of the dermis of 4 patients with localized and 6 with systemic scleroderma revealed numerous fine elastic fibrils in areas of fibrosis. Immunofluorescence and immunoelectron microscopy were performed with antibodies against fibrillin and amorphous elastin. The lower dermis revealed an increase in 10-nm microfibrils interspersed between collagen fibrils. These microfibrils stained for fibrillin but not for amorphous elastin. Fibrosis in localized and systemic scleroderma involves the deposition of collagen fibrils and microfibrils.
Insights
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.
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.
- 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.