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Erdheim-chester disease
D Murray1, M Marshall, E England
1Department of Diagnostic Radiology, Royal Perth Hospital, Perth, WA, Australia.
Insights
Erdheim-Chester disease (ECD) is a rare condition causing bone sclerosis and infiltration. This study highlights ECD
Area of Science:
- Histiocytic Disorders
- Rare Diseases
- Radiology
Background:
- Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytic disorder.
- Characterized by systemic infiltration, often affecting retroperitoneal and renal areas.
- Long bone sclerosis is a hallmark radiographic feature.
Observation:
- Two confirmed cases of ECD presented with non-malignant retroperitoneal and peri-renal infiltration.
- This infiltration led to dilatation of the upper urinary tracts.
- Radiographic findings included typical long bone sclerosis, confirmed by CT and MRI.
Findings:
- Imaging studies demonstrated characteristic retroperitoneal and peri-renal involvement in ECD.
- The observed infiltration caused significant upper urinary tract dilatation.
- Long bone sclerosis was a consistent finding across both cases.
Implications:
- ECD should be considered in the differential diagnosis of non-malignant retroperitoneal infiltration.
- Radiographic evaluation of long bones is crucial for diagnosing ECD.
- Conservative management was employed in both presented cases.
Abstract:
Erdheim-Chester disease (ECD) is a rare histiocytic infiltrative disease, characterized by long bone sclerosis. We describe two confirmed cases of ECD, both of which demonstrate non-malignant retroperitoneal and peri-renal infiltration causing dilatation of the upper renal tracts. The cases are illustrated with contrast studies, computed tomography (CT) and magnetic resonance imaging (MRI). Typical sclerosis of the long bones was apparent on radiography. Both cases have been treated conservatively to date.A brief review of the literature regarding the manifestations of ECD is included. In cases of non-malignant retroperitoneal infiltration, ECD should be considered as a diagnosis and radiographs of the long bones performed.