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Cutaneous amyloidosis in patients with progressive systemic sclerosis
1Department of Dermatology, Nagoya University School of Medicine, Japan.
Cutis
|January 1, 1996
Summary
Amyloidosis is a rare complication of progressive systemic sclerosis (PSS). This study found localized cutaneous amyloid deposits in 9% of PSS patients, suggesting a potential link between PSS and skin amyloidosis.
Area of Science:
- Rheumatology and Dermatology
- Pathology and Medical Research
Background:
- Amyloidosis is infrequently reported as a complication of progressive systemic sclerosis (PSS).
- Cutaneous manifestations are common in PSS, but specific amyloid deposition is rare.
Purpose of the Study:
- To investigate the occurrence and characteristics of amyloidosis in patients with progressive systemic sclerosis (PSS).
- To determine the type and extent of amyloid deposits in the skin of PSS patients.
Main Methods:
- Histochemical and electron microscopic examinations of skin biopsies.
- Morphologic and immunologic studies to classify amyloid deposits.
- Assessment of serum amyloid A levels and PSS disease types (Barnett classification).
Main Results:
- Amyloid deposits were identified in the skin of 6 out of 66 PSS patients (9%).
- The amyloid deposits were characterized as localized cutaneous type.
- Serum amyloid A levels were normal or moderately elevated; no severe visceral involvement was noted.
Conclusions:
- Cutaneous amyloidosis can occur as a complication of progressive systemic sclerosis (PSS).
- The observed amyloidosis in PSS patients is typically localized to the skin.
- Further research is warranted to understand the pathogenesis and clinical significance of PSS-associated amyloidosis.