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Nephrogenic fibrosing dermopathy: the first 6 years
1Dermatopathology Service, Yale University, New Haven, Connecticut, USA. shawn.cowper@yale.edu
Current Opinion in Rheumatology
|October 22, 2003
Summary
Nephrogenic fibrosing dermopathy (NFD) is a skin condition linked to kidney insufficiency. Research suggests circulating fibrocytes may cause NFD, offering new diagnostic and therapeutic avenues.
Area of Science:
- Dermatology
- Nephrology
- Pathogenesis of fibrotic disorders
Background:
- Nephrogenic fibrosing dermopathy (NFD) is a recently identified scleroderma-like fibrotic skin condition.
- NFD develops in patients experiencing renal insufficiency.
Purpose of the Study:
- To review recent case reports on NFD.
- To examine theories regarding the pathogenesis of NFD.
- To provide a framework for understanding NFD based on registry data and published cases.
Main Methods:
- Review of published case reports.
- Analysis of data from the Yale University NFD Registry Project.
- Dual immunohistochemical staining for CD34 and procollagen.
Main Results:
- Recent studies have clarified the clinical spectrum, epidemiology, and pathogenesis of NFD.
- Yellow scleral plaques and antiphospholipid antibodies are proposed NFD markers.
- Epidemiologic data reveal distinct patterns of NFD onset.
- Dual staining suggests NFD dermal cells may be circulating fibrocytes.
Conclusions:
- Scenario classification may aid in identifying NFD triggers and predicting prognosis.
- Dual immunolabeling shows potential as a sensitive and specific diagnostic method for NFD.
- NFD may be a systemic disorder involving aberrant circulating fibroblast precursor fibrocytes.