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Case report
peer-reviewed

A Case of Incidentally-diagnosed Erdheim-Chester Disease



Abstract

Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytosis that may be clonal and inflammatory in origin. The hallmark of the disease is infiltration of various organ systems by CD68+/CD1a- histiocytes containing foamy lipid-laden inclusions. The manifestations and course of the disease are variable and depend on the organ systems that are affected. Patients may be asymptomatic or may develop life-threatening complications, including myocardial infarction. The most common clinical manifestation is lower extremity bone pain. Imaging manifestations of the disease include symmetric osteosclerosis of the distal long bones, circumferentially “coated” aorta, pleural and pericardial thickening/fluid, and perirenal encasement. Treatment for the disease is evolving, particularly with the use of molecular BRAF inhibition. We present a case of a patient with ECD initially suspected based on the imaging manifestations.



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Case report
peer-reviewed

A Case of Incidentally-diagnosed Erdheim-Chester Disease


Author Information

Atman A. Dave Corresponding Author

Medical Education, Saint Luke’s Hospital of Kansas City

Susan E. Gutschow

Department of Radiology, Saint Luke’s Hospital of Kansas City

Christopher M. Walker

Department of Radiology, Saint Luke’s Hospital of Kansas City


Ethics Statement and Conflict of Interest Disclosures

Human subjects: Consent was obtained by all participants in this study. Conflicts of interest: The authors have declared that no conflicts of interest exist.


Case report
peer-reviewed

A Case of Incidentally-diagnosed Erdheim-Chester Disease


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Case report
peer-reviewed

A Case of Incidentally-diagnosed Erdheim-Chester Disease

  • Author Information
    Atman A. Dave Corresponding Author

    Medical Education, Saint Luke’s Hospital of Kansas City

    Susan E. Gutschow

    Department of Radiology, Saint Luke’s Hospital of Kansas City

    Christopher M. Walker

    Department of Radiology, Saint Luke’s Hospital of Kansas City


    Ethics Statement and Conflict of Interest Disclosures

    Human subjects: Consent was obtained by all participants in this study. Conflicts of interest: The authors have declared that no conflicts of interest exist.

    Acknowledgements


    Article Information

    Published: September 13, 2016

    DOI

    10.7759/cureus.781

    Cite this article as:

    Dave A A, Gutschow S E, Walker C M (September 13, 2016) A Case of Incidentally-diagnosed Erdheim-Chester Disease. Cureus 8(9): e781. doi:10.7759/cureus.781

    Publication history

    Received by Cureus: August 01, 2016
    Peer review began: August 11, 2016
    Peer review concluded: September 07, 2016
    Published: September 13, 2016

    Copyright

    © Copyright 2016
    Dave et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 3.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

    License

    This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytosis that may be clonal and inflammatory in origin. The hallmark of the disease is infiltration of various organ systems by CD68+/CD1a- histiocytes containing foamy lipid-laden inclusions. The manifestations and course of the disease are variable and depend on the organ systems that are affected. Patients may be asymptomatic or may develop life-threatening complications, including myocardial infarction. The most common clinical manifestation is lower extremity bone pain. Imaging manifestations of the disease include symmetric osteosclerosis of the distal long bones, circumferentially “coated” aorta, pleural and pericardial thickening/fluid, and perirenal encasement. Treatment for the disease is evolving, particularly with the use of molecular BRAF inhibition. We present a case of a patient with ECD initially suspected based on the imaging manifestations.



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Atman A. Dave, Medical Student

Medical Education, Saint Luke’s Hospital of Kansas City

For correspondence:
atman.dave@gmail.com

Susan E. Gutschow

Department of Radiology, Saint Luke’s Hospital of Kansas City

Christopher M. Walker

Department of Radiology, Saint Luke’s Hospital of Kansas City

Atman A. Dave, Medical Student

Medical Education, Saint Luke’s Hospital of Kansas City

For correspondence:
atman.dave@gmail.com

Susan E. Gutschow

Department of Radiology, Saint Luke’s Hospital of Kansas City

Christopher M. Walker

Department of Radiology, Saint Luke’s Hospital of Kansas City