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Systemic sclerosis with multiple nodules: characterization of the extracellular matrix
P Moinzadeh1, P Agarwal, W Bloch
1Department of Dermatology, University of Cologne, Germany. pia.moinzadeh@uk-koeln.de
Archives of Dermatological Research
|July 10, 2013
Summary
Systemic sclerosis (SSc) can cause rare nodular scleroderma. Cartilage Oligomeric Matrix Protein (COMP) excess in nodules, similar to keloids, may drive fibroblast activation and fibrosis.
Area of Science:
- Dermatology
- Rheumatology
- Pathology
Background:
- Systemic sclerosis (SSc) is a fibrotic disease with unknown etiology.
- Pathophysiology involves vascular, immunological, and fibrotic processes.
Observation:
- A patient with diffuse SSc (dSSc) developed multiple subcutaneous nodules.
- Histologic evaluation confirmed nodular scleroderma, a rare condition.
Findings:
- Nodules showed high-density collagen deposition, with immature fibrils.
- Increased Cartilage Oligomeric Matrix Protein (COMP), collagen XII, and fibrillin-1 were observed.
- Nodules lacked myofibroblasts, resembling keloids.
Implications:
- COMP's role in reorganizing collagen and potentially activating fibroblasts via TGFβ suggests a fibrotic mechanism.
- This may explain sustained fibroblast activation and extracellular matrix production in SSc nodules.
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