Degos' disease with constrictive pericarditis: a case report

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K Yukiiri1, K Mizushige, T Ueda

  • 1Second Department of Internal Medicine, Kagawa Medical University, Japan.

Japanese Circulation Journal
|June 30, 2000
PubMed

Insights

Degos disease can cause constrictive pericarditis, leading to left ventricular dysfunction and wall motion abnormalities. This study highlights the cardiac implications of this rare condition.

Area of Science:

  • Cardiology
  • Rare Diseases
  • Vascular Medicine

Background:

  • Degos disease is a rare systemic vasculopathy.
  • Cardiac involvement in Degos disease is not well-characterized.
  • Understanding cardiac manifestations is crucial for patient management.

Observation:

  • A 47-year-old male patient with Degos disease presented with apical left ventricular hypokinesis and pericardial effusion.
  • Echocardiography revealed impaired left ventricular wall motion.
  • Pericardial effusion was noted on cardiac imaging.

Findings:

  • 201Tl scintigraphy and intracoronary Doppler flowmetry assessed myocardial perfusion and coronary flow reserve.
  • Coronary flow reserve was normal, with no significant angiographical coronary stenosis.
  • A pressure study indicated constrictive dysfunction of both ventricles.

Implications:

  • Constrictive pericarditis, potentially induced by pericardial vasculitis, is suggested as the cause.
  • This constrictive pericarditis may underlie the observed left ventricular wall motion abnormality.
  • The findings underscore the importance of cardiac evaluation in Degos disease.

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