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Calcific constrictive pericarditis in Degos' disease
Insights
Degos disease, a rare vasculitis, can cause constrictive pericarditis. This case report details extensive pericardial calcification, a previously undocumented finding in this condition.
Area of Science:
- Cardiology
- Rheumatology
- Dermatology
Background:
- Degos disease is a rare systemic vasculitis.
- It typically presents with characteristic skin and gastrointestinal lesions.
- Pericardial involvement is uncommon in Degos disease.
Observation:
- A 31-year-old woman with a history of Degos disease presented with constrictive pericarditis.
- Chest roentgenogram revealed extensive pericardial calcification.
- This finding is unusual given the known manifestations of Degos disease.
Findings:
- Constrictive pericarditis is a rare complication of Degos disease.
- Extensive pericardial calcification has not been previously reported in Degos disease.
- This case highlights an unusual presentation of a rare disease.
Implications:
- This case expands the spectrum of cardiovascular complications associated with Degos disease.
- It suggests the need for heightened awareness of cardiac involvement in patients with Degos disease.
- Further research may elucidate the mechanisms linking Degos disease to pericardial calcification and constriction.
Abstract:
A 31-year-old woman presented with symptoms and signs of constrictive pericarditis. She had a history of Degos' disease, a rare disorder characterized by skin and bowel lesions thought to be secondary to vasculitis. A chest roentgenogram showed extensive calcification of the pericardium. Although pleural and pericardial involvement has been reported in this disease, constrictive pericarditis is most unusual, and radiographically demonstrable calcification of the pericardium has not been reported previously.