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Mast cell changes in a case of rapidly progressive scleroderma-ultrastructural analysis
1Department of Medicine, University of Colorado School of Medicine, Denver 80262.
The Journal of Investigative Dermatology
|February 1, 1989
Summary
Mast cells actively participate in acute scleroderma, a severe autoimmune disease. Electron microscopy revealed activated mast cells in affected skin, suggesting their role in disease progression.
Area of Science:
- Dermatology
- Immunology
- Pathology
Background:
- Scleroderma is a connective tissue disease characterized by skin hardening and tightening.
- Rapidly progressive scleroderma can lead to severe morbidity and mortality.
- The role of mast cells in the pathogenesis of scleroderma remains incompletely understood.
Observation:
- A 63-year-old woman with rapidly progressive scleroderma experienced significant itching.
- Electron microscopy of unaffected skin showed normal mast cells.
- Mast cells in affected skin exhibited signs of activation, including degranulation and altered cytoplasmic composition.
Findings:
- Mast cells in scleroderma lesions displayed a spectrum of activation, with reduced granule content and increased polysomes/mitochondria.
- Evidence of transgranulation, where fibroblasts may acquire mast cell granules, was observed.
- These findings suggest mast cell degranulation and activation are prominent features in acute scleroderma.
Implications:
- Mast cells may play a direct role in the inflammatory and fibrotic processes of acute scleroderma.
- Targeting mast cell activation could be a potential therapeutic strategy for scleroderma.
- Further research is warranted to elucidate the precise mechanisms of mast cell involvement in scleroderma pathogenesis.