Erdheim-Chester disease with interatrial septum involvement

Area of Science:

• Histiocytosis
• Radiology
• Cardiovascular Imaging

Background:

• Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis.
• Diagnosis typically follows established radiologic findings after pathological confirmation.
• Systemic manifestations are common, but cardiac involvement is less frequently highlighted.

Observation:

• A patient presented with symptoms suggestive of Erdheim-Chester disease without a prior diagnosis.
• Advanced imaging modalities including CT, MRI, and PET were utilized.
• Unusual findings included interatrial septum and diffuse cardiac involvement.

Findings:

• Radiologic imaging revealed characteristic Erdheim-Chester disease lesions.
• The imaging demonstrated significant interatrial septum and diffuse cardiac involvement.
• This presentation expanded the known spectrum of Erdheim-Chester disease manifestations.

Implications:

• Highlights the importance of considering Erdheim-Chester disease in cases with unexplained cardiac and retroperitoneal findings.
• Suggests that cardiac and interatrial septum imaging should be part of the diagnostic workup for Erdheim-Chester disease.
• Broadens the differential diagnosis for radiologists evaluating lesions in the interatrial septum and retroperitoneum.