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Progressive Diaphyseal Dysplasia
Cheryl A. Petersilge, MD, MBA; Nancy A. Chauvin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Progressive diaphyseal dysplasia (PDD)
            • Synonyms

              • Engelmann-Camurati disease (ECD)
              • Camurati-Engelmann disease/syndrome (CED)
            • Definitions

              • Belongs to group of craniotubular hyperostoses
              • Sclerosing bone dysplasia

            IMAGING

            • General Features

              • Imaging Recommendations

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Nuclear Medicine Findings

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Rossi M et al: Looking for new anabolic treatment from rare diseases of bone formation. J Endocrinol. 248(2):R29-40, 2021
                                      2. Ayyavoo A et al: Elimination of pain and improvement of exercise capacity in Camurati-Engelmann disease with losartan. J Clin Endocrinol Metab. 99(11):3978-82, 2014
                                      3. Harisankar CN et al: Tc99m-MDP bone scintigraphy in Engelmann-Camurati disease. Indian J Nucl Med. 26(1):44-5, 2011
                                      4. Ihde LL et al: Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics. 31(7):1865-82, 2011
                                      5. DamiĆ” Ade B et al: Bone scintigraphy in Engelmann-Camurati disease. Clin Nucl Med. 35(7):559-60, 2010
                                      6. Bartuseviciene A et al: Camurati-Engelmann disease: imaging, clinical features and differential diagnosis. Skeletal Radiol. 38(11):1037-43, 2009
                                      7. Janssens K et al: Camurati-Engelmann disease: review of the clinical, radiological, and molecular data of 24 families and implications for diagnosis and treatment. J Med Genet. 43(1):1-11, 2006
                                      8. Kaftori JK et al: Progressive diaphyseal dysplasia (Camurati-Engelmann): radiographic follow-up and CT findings. Radiology. 164(3):777-82, 1987
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Progressive diaphyseal dysplasia (PDD)
                                                • Synonyms

                                                  • Engelmann-Camurati disease (ECD)
                                                  • Camurati-Engelmann disease/syndrome (CED)
                                                • Definitions

                                                  • Belongs to group of craniotubular hyperostoses
                                                  • Sclerosing bone dysplasia

                                                IMAGING

                                                • General Features

                                                  • Imaging Recommendations

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Nuclear Medicine Findings

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Rossi M et al: Looking for new anabolic treatment from rare diseases of bone formation. J Endocrinol. 248(2):R29-40, 2021
                                                                          2. Ayyavoo A et al: Elimination of pain and improvement of exercise capacity in Camurati-Engelmann disease with losartan. J Clin Endocrinol Metab. 99(11):3978-82, 2014
                                                                          3. Harisankar CN et al: Tc99m-MDP bone scintigraphy in Engelmann-Camurati disease. Indian J Nucl Med. 26(1):44-5, 2011
                                                                          4. Ihde LL et al: Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis. Radiographics. 31(7):1865-82, 2011
                                                                          5. DamiĆ” Ade B et al: Bone scintigraphy in Engelmann-Camurati disease. Clin Nucl Med. 35(7):559-60, 2010
                                                                          6. Bartuseviciene A et al: Camurati-Engelmann disease: imaging, clinical features and differential diagnosis. Skeletal Radiol. 38(11):1037-43, 2009
                                                                          7. Janssens K et al: Camurati-Engelmann disease: review of the clinical, radiological, and molecular data of 24 families and implications for diagnosis and treatment. J Med Genet. 43(1):1-11, 2006
                                                                          8. Kaftori JK et al: Progressive diaphyseal dysplasia (Camurati-Engelmann): radiographic follow-up and CT findings. Radiology. 164(3):777-82, 1987