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Erdheim-Chester Disease
B.J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Erdheim-Chester (EC)
          • Synonyms

            • Lipogranulomatosis, lipoid granulomatosis, lipid (cholesterol) granulomatosis, polyostotic sclerosing histiocytosis
          • Definitions

            • Histiocytosis characterized by infiltration of skeleton and viscera by lipid-laden histiocytes
              • Leads to fibrosis and osteosclerosis

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    Selected References

                                    1. Campochiaro C et al: Erdheim-Chester disease. Eur J Intern Med. ePub, 2015
                                    2. Bindra J et al: Erdheim-Chester disease: an unusual presentation of an uncommon disease. Skeletal Radiol. 43(6):835-40, 2014
                                    3. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                    4. Drier A et al: Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology. 255(2):586-94, 2010
                                    5. Arnaud L et al: 18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester disease. Arthritis Rheum. 60(10):3128-38, 2009
                                    6. De Filippo M et al: Erdheim-Chester disease: clinical and radiological findings. Radiol Med. 114(8):1319-1329, 2009
                                    7. Tran TA et al: Erdheim-Chester disease in childhood: a challenging diagnosis and treatment. J Pediatr Hematol Oncol. 31(10):782-6, 2009
                                    8. Zanglis A et al: Erdheim-Chester disease: symmetric uptake in the (99m)Tc-MDP bone scan. Hell J Nucl Med. 11(3):164-7, 2008
                                    9. Srikulmontree T et al: Treatment of skeletal Erdheim-Chester disease with zoledronic acid: case report and proposed mechanisms of action. Rheumatol Int. 27(3):303-7, 2007
                                    10. Dion E et al: Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement. Radiology. 238(2):632-9, 2006
                                    11. Braiteh F et al: Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. Blood. 106(9):2992-4, 2005
                                    12. De Abreu MR et al: Erdheim-Chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement. AJNR Am J Neuroradiol. 25(4):627-30, 2004
                                    13. Ivan D et al: Erdheim-Chester disease: a unique presentation with liver involvement and vertebral osteolytic lesions. Arch Pathol Lab Med. 127(8):e337-9, 2003
                                    14. Klieger MR et al: Erdheim-Chester disease: a unique presentation with multiple osteolytic lesions of the spine and pelvis that spared the appendicular skeleton. AJR Am J Roentgenol. 178(2):429-32, 2002
                                    15. Vinh TN et al: Erdheim-Chester disease. Fletcher CDM et al: World Health Organization Classification of Tumors: Tumours of Soft tissue and Bone. Lyon: IARC Press. 347, 2002
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Erdheim-Chester (EC)
                                            • Synonyms

                                              • Lipogranulomatosis, lipoid granulomatosis, lipid (cholesterol) granulomatosis, polyostotic sclerosing histiocytosis
                                            • Definitions

                                              • Histiocytosis characterized by infiltration of skeleton and viscera by lipid-laden histiocytes
                                                • Leads to fibrosis and osteosclerosis

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT Findings

                                                  • MR Findings

                                                    • Nuclear Medicine Findings

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • Gross Pathologic & Surgical Features

                                                          • Microscopic Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Consider

                                                                      Selected References

                                                                      1. Campochiaro C et al: Erdheim-Chester disease. Eur J Intern Med. ePub, 2015
                                                                      2. Bindra J et al: Erdheim-Chester disease: an unusual presentation of an uncommon disease. Skeletal Radiol. 43(6):835-40, 2014
                                                                      3. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                                                      4. Drier A et al: Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology. 255(2):586-94, 2010
                                                                      5. Arnaud L et al: 18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester disease. Arthritis Rheum. 60(10):3128-38, 2009
                                                                      6. De Filippo M et al: Erdheim-Chester disease: clinical and radiological findings. Radiol Med. 114(8):1319-1329, 2009
                                                                      7. Tran TA et al: Erdheim-Chester disease in childhood: a challenging diagnosis and treatment. J Pediatr Hematol Oncol. 31(10):782-6, 2009
                                                                      8. Zanglis A et al: Erdheim-Chester disease: symmetric uptake in the (99m)Tc-MDP bone scan. Hell J Nucl Med. 11(3):164-7, 2008
                                                                      9. Srikulmontree T et al: Treatment of skeletal Erdheim-Chester disease with zoledronic acid: case report and proposed mechanisms of action. Rheumatol Int. 27(3):303-7, 2007
                                                                      10. Dion E et al: Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement. Radiology. 238(2):632-9, 2006
                                                                      11. Braiteh F et al: Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. Blood. 106(9):2992-4, 2005
                                                                      12. De Abreu MR et al: Erdheim-Chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement. AJNR Am J Neuroradiol. 25(4):627-30, 2004
                                                                      13. Ivan D et al: Erdheim-Chester disease: a unique presentation with liver involvement and vertebral osteolytic lesions. Arch Pathol Lab Med. 127(8):e337-9, 2003
                                                                      14. Klieger MR et al: Erdheim-Chester disease: a unique presentation with multiple osteolytic lesions of the spine and pelvis that spared the appendicular skeleton. AJR Am J Roentgenol. 178(2):429-32, 2002
                                                                      15. Vinh TN et al: Erdheim-Chester disease. Fletcher CDM et al: World Health Organization Classification of Tumors: Tumours of Soft tissue and Bone. Lyon: IARC Press. 347, 2002