Cardiac Erdheim-Chester

Driss Bassou, Abdenacer El Kharras, Taoufik Amezyane T, Hassan En Nouali, Mohamed Elbaaj, Mohamed Benameur, Abdellatif Darbi

Internal Medicine · 2009 · 19 citations · 1 references

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Abstract

A 59-year-old woman presented with an 8-month history of neurologic disturbance. On examination, she had eyelid xantelasmas, dyspnea and diminished heart sounds. Chest Xray showed cardiomegaly. Pericardial effusion was confirmed on two-dimensional echocardiogram. Laboratory findings revealed an elevated erythrocyte sedimentation rate. Contrast-enhanced MDCT scan of the chest showed on axial reformation, pericardial and pleural effusion ( Picture 1A, stars) and infiltration of the atrioventricular groove and right atrial wall as soft-tissue pseudomass of low intensity (Picture 1A, arrow). Immunohistochemistry of the pericardial biopsy demonstrated the presence of non-Langerhans histiocytes markers, positivity for CD68, and negativity for CD1a and PS-100 protein, confirming the diagnosis of ECD (Picture 1B). Anteroposterior radioagraphs of two legs shows pathognomonic bilateral symmetrical diametaphyseal

References

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