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