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Erdheim-Chester Disease
Jonathan Hero Chung, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Erdheim-Chester disease (ECD)
            • Definitions

              • Non-Langerhans cell histiocytosis of unknown origin

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Haroche J et al: Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients. Circulation. 119(25):e597-8, 2009
                                    2. Allen TC et al: Pulmonary and ophthalmic involvement with Erdheim-Chester disease: a case report and review of the literature. Arch Pathol Lab Med. 128(12):1428-31, 2004
                                    3. Dion E et al: Imaging of thoracoabdominal involvement in Erdheim-Chester disease. AJR Am J Roentgenol. 183(5):1253-60, 2004
                                    4. Kenn W et al: Erdheim-Chester disease: evidence for a disease entity different from Langerhans cell histiocytosis? Three cases with detailed radiological and immunohistochemical analysis. Hum Pathol. 31(6):734-9, 2000
                                    5. Rush WL et al: Pulmonary pathology of Erdheim-Chester disease. Mod Pathol. 13(7):747-54, 2000
                                    6. Wittenberg KH et al: Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings. AJR Am J Roentgenol. 174(5):1327-31, 2000
                                    7. Egan AJ et al: Erdheim-Chester disease: clinical, radiologic, and histopathologic findings in five patients with interstitial lung disease. Am J Surg Pathol. 23(1):17-26, 1999
                                    8. Veyssier-Belot C et al: Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine (Baltimore). 75(3):157-69, 1996
                                    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

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Erdheim-Chester disease (ECD)
                                              • Definitions

                                                • Non-Langerhans cell histiocytosis of unknown origin

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • Gross Pathologic & Surgical Features

                                                          • Microscopic Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Haroche J et al: Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients. Circulation. 119(25):e597-8, 2009
                                                                      2. Allen TC et al: Pulmonary and ophthalmic involvement with Erdheim-Chester disease: a case report and review of the literature. Arch Pathol Lab Med. 128(12):1428-31, 2004
                                                                      3. Dion E et al: Imaging of thoracoabdominal involvement in Erdheim-Chester disease. AJR Am J Roentgenol. 183(5):1253-60, 2004
                                                                      4. Kenn W et al: Erdheim-Chester disease: evidence for a disease entity different from Langerhans cell histiocytosis? Three cases with detailed radiological and immunohistochemical analysis. Hum Pathol. 31(6):734-9, 2000
                                                                      5. Rush WL et al: Pulmonary pathology of Erdheim-Chester disease. Mod Pathol. 13(7):747-54, 2000
                                                                      6. Wittenberg KH et al: Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings. AJR Am J Roentgenol. 174(5):1327-31, 2000
                                                                      7. Egan AJ et al: Erdheim-Chester disease: clinical, radiologic, and histopathologic findings in five patients with interstitial lung disease. Am J Surg Pathol. 23(1):17-26, 1999
                                                                      8. Veyssier-Belot C et al: Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine (Baltimore). 75(3):157-69, 1996