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Dermal mast cell degranulation in systemic sclerosis.
J R Seibold1, R C Giorno, H N Claman
1Division of Rheumatology, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School, New Brunswick 08903-0019.
Arthritis and Rheumatism
|November 1, 1990
Summary
Mast cells increase and degranulate in systemic sclerosis (SSc) skin, preceding fibrosis. This suggests mast cells play a role in SSc skin progression, especially in early, active disease.
Area of Science:
- Dermatology
- Immunology
- Pathology
Background:
- Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by skin thickening and fibrosis.
- The role of mast cells in the pathogenesis of SSc skin manifestations is not fully understood.
Purpose of the Study:
- To investigate the number and activation status of mast cells in the skin of patients with generalized SSc.
- To determine if mast cell changes correlate with disease activity and progression.
Main Methods:
- Paired skin biopsy samples (involved and uninvolved) were collected from 19 SSc patients (11 early, 8 late disease).
- Biopsies were double-stained to quantify total and degranulated mast cells.
Main Results:
- Mast cell numbers were elevated in both involved and uninvolved skin across early and late SSc.
- Degranulated mast cells increased in involved skin (early and late) and early uninvolved skin.
- No mast cell degranulation was observed in previously involved, now normal skin of late-stage patients.
Conclusions:
- Increased mast cell numbers and degranulation occur early in SSc skin, preceding dermal fibrosis.
- Mast cell degranulation appears linked to active disease progression and may not be present in resolving lesions.
- These findings suggest a significant role for mast cells in the clinical progression of SSc skin changes.