Erdheim-Chester disease

Case contributed by Dr Clarissa Valle

Presentation

Middle aged woman hospitalized because of an acute chest pain. History of non-X histiocytosis (only skin involvement), chronic kidney disease and left ventricular diastolic dysfunction with left atrial stiffness.

Patient Data

Age: 56
Gender: Female
CT

CT examination performed in the ER department in the suspicion of aortic dissection showing large pericardial effusion.

X-ray

Because of a lower extremities pain during her hospitalization, a hip and bilateral femur x-ray were performed.
Findings: bilateral, symmetric metaphyseal and diaphyseal sclerosis of femurs.
No periosteal reaction.

Conclusive suspicion of Erdheim-Chester disease.

Case Discussion

Diagnosis of Erdheim-Chester disease considered certain both because of the almost unequivocal and pathognomic x-ray appearance and because of the known history of non-X histiocytosis (although considered with the only skin involvement).

Moreover, the clinical presentation with acute chest pain and the pericardial finding agree with the diagnosis of E-C disease. 

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Case information

rID: 68398
Published: 28th Jun 2019
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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