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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Erdheim-Chester disease (ECD)
Synonyms
- Lipogranulomatosis, lipoid granulomatosis, lipid (cholesterol) granulomatosis, polyostotic sclerosing histiocytosis
Definitions
- Rare histiocytosis characterized by infiltration of skeleton and viscera by lipid-laden histiocytes
- Leads to fibrosis and bone sclerosis
- Because Langerhans cell histiocytosis is more common than all other histiocytoses combined, ECD is grouped with non-Langerhans histiocytoses
- Rare histiocytosis characterized by infiltration of skeleton and viscera by lipid-laden histiocytes
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
Gross Pathologic & Surgical Features
Microscopic Features
Genetics
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Choraria A et al: Musculoskeletal imaging features of non-Langerhans cell histiocytoses. Skeletal Radiol. 50(10):1921-40, 2021
- Chung AD et al: Primary and secondary diseases of the perinephric space: an approach to imaging diagnosis with emphasis on MRI. Clin Radiol. 76(1):75.e13-26, 2021
- Garg N et al: Clinical and neuroimaging manifestations of Erdheim-Chester disease: a review. J Neuroimaging. 31(1):35-44, 2021
- Salama HA et al: Highlights of the management of adult histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease, and hemophagocytic lymphohistiocytosis. Clin Lymphoma Myeloma Leuk. 21(1):e66-75, 2020
- Kumar P et al: Imaging findings in Erdheim-Chester disease: what every radiologist needs to know. Pol J Radiol. 83:e54-62, 2018
- Triffo WJ et al: The "hairy kidney" sign. Abdom Radiol (NY). 42(3):979-80, 2017
- White TV et al: Non-sclerotic bone involvement in Erdheim-Chester: PET/CT and MRI findings in a 15-year-old boy. Pediatr Radiol. 46(9):1345-9, 2016
- Campochiaro C et al: Erdheim-Chester disease. Eur J Intern Med. 26(4):223-9, 2015
- Bindra J et al: Erdheim-Chester disease: an unusual presentation of an uncommon disease. Skeletal Radiol. 43(6):835-40, 2014
- Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
- Drier A et al: Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology. 255(2):586-94, 2010
- 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
- De Filippo M et al: Erdheim-Chester disease: clinical and radiological findings. Radiol Med. 114(8):1319-29, 2009
- Tran TA et al: Erdheim-Chester disease in childhood: a challenging diagnosis and treatment. J Pediatr Hematol Oncol. 31(10):782-6, 2009
- 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
- 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
- Dion E et al: Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement. Radiology. 238(2):632-9, 2006
- Braiteh F et al: Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. Blood. 106(9):2992-4, 2005
- 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
- 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
- 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
- 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
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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Erdheim-Chester disease (ECD)
Synonyms
- Lipogranulomatosis, lipoid granulomatosis, lipid (cholesterol) granulomatosis, polyostotic sclerosing histiocytosis
Definitions
- Rare histiocytosis characterized by infiltration of skeleton and viscera by lipid-laden histiocytes
- Leads to fibrosis and bone sclerosis
- Because Langerhans cell histiocytosis is more common than all other histiocytoses combined, ECD is grouped with non-Langerhans histiocytoses
- Rare histiocytosis characterized by infiltration of skeleton and viscera by lipid-laden histiocytes
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
Gross Pathologic & Surgical Features
Microscopic Features
Genetics
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Choraria A et al: Musculoskeletal imaging features of non-Langerhans cell histiocytoses. Skeletal Radiol. 50(10):1921-40, 2021
- Chung AD et al: Primary and secondary diseases of the perinephric space: an approach to imaging diagnosis with emphasis on MRI. Clin Radiol. 76(1):75.e13-26, 2021
- Garg N et al: Clinical and neuroimaging manifestations of Erdheim-Chester disease: a review. J Neuroimaging. 31(1):35-44, 2021
- Salama HA et al: Highlights of the management of adult histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease, and hemophagocytic lymphohistiocytosis. Clin Lymphoma Myeloma Leuk. 21(1):e66-75, 2020
- Kumar P et al: Imaging findings in Erdheim-Chester disease: what every radiologist needs to know. Pol J Radiol. 83:e54-62, 2018
- Triffo WJ et al: The "hairy kidney" sign. Abdom Radiol (NY). 42(3):979-80, 2017
- White TV et al: Non-sclerotic bone involvement in Erdheim-Chester: PET/CT and MRI findings in a 15-year-old boy. Pediatr Radiol. 46(9):1345-9, 2016
- Campochiaro C et al: Erdheim-Chester disease. Eur J Intern Med. 26(4):223-9, 2015
- Bindra J et al: Erdheim-Chester disease: an unusual presentation of an uncommon disease. Skeletal Radiol. 43(6):835-40, 2014
- Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
- Drier A et al: Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology. 255(2):586-94, 2010
- 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
- De Filippo M et al: Erdheim-Chester disease: clinical and radiological findings. Radiol Med. 114(8):1319-29, 2009
- Tran TA et al: Erdheim-Chester disease in childhood: a challenging diagnosis and treatment. J Pediatr Hematol Oncol. 31(10):782-6, 2009
- 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
- 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
- Dion E et al: Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement. Radiology. 238(2):632-9, 2006
- Braiteh F et al: Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. Blood. 106(9):2992-4, 2005
- 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
- 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
- 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
- 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
