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Erdheim-Chester disease
Erdheim-Chester disease (ECD) is a rare non-Langerhan's cell, non-familial granulomatosis, with widespread manifestations and of highly variable severity 1.
Erdheim-Chester disease is a rare, non-inherited disease of middle age with a slight male predominance 6.
Clinical presentation
Patients may present with a focal symptoms through to multi-system involvement and failure 5. The most common presenting symptom is bone pain. Patients may also present with focal neurological signs, exophthalmos, retroperitoneal fibrosis, diabetes insipidus, and dyspnoea due to extra-skeletal involvement of these systems.
Erdheim-Chester disease is a systemic lipogranulomatous disorder with infiltration by lipid-laden histiocytes (foamy macrophages), Touton giant cells and a variable amount of background fibrosis 2. In contrast to histiocytosis X no S-100 nor CD1 are detected 1.
Radiographic features
Musculoskeletal involvement is most common, with multifocal extraskeletal involvement seen in 30-50% of patients 1,2.
Skeletal involvement
- bilateral, symmetric metaphyseal and diaphyseal sclerosis 1,2
- increased uptake on Tc-MDP bone scan 7
- cortical thickening
lung - see pulmonary manifestations of Erdheim-Chester disease
- can appear similar to Langerhan's Cell Histiocytosis with predominantly cystic disease, septal thickening and preserved lung volume
- chest radiographs will often show interstitial oedema pattern (interlobular septal thickening) with cardiomegaly and pleural effusions that do not respond to diuretics
kidneys and retroperitoneum
- often involved
- usually asymptomatic 1
- inferior vena cava and pelvic ureters are typically spared, which are useful cross-sectional imaging findings for differentiation of retroperitoneal Erdheim-Chester disease from retroperitoneal fibrosis 8
- skin
- retro-orbital tissue
- retrograde extension along the optic nerve to the hypothalamus may explain the distribution of brain involvement 4
- heart, pericardium and aorta 2
Intracranial involvement of the dura, brain and pituitary are rare 3:
- dural accumulations, may mimic meningiomas, with enhancing soft tissue masses
- T2 signal characteristics are somewhat different, as the accumulations in ECD are hypointense 3
- brain: usually the hypothalamus 3
- pituitary infundibulum: presenting with diabetes insipidus
Treatment and prognosis
Steroids, radiotherapy and chemotherapy have all been used but with little effect, with some patients relentlessly progressing 1. Pulmonary fibrosis and cardiac failure are the most common causes of death 2. Given the small volume of published data, mortality rates are sketchy, but may be as high as 60% 2.
Surgical or percutaneous intervention for hydronephrosis, orbital or meningeal involvement is useful for symptomatic local disease.
- 1. Verdalles U, Goicoechea M, García de Vinuesa S et-al. Erdheim-Chester disease: a rare cause of renal failure. Nephrol. Dial. Transplant. 2007;22 (6): 1776-7. doi:10.1093/ndt/gfm115 - Pubmed citation
- 2. Myra C, Sloper L, Tighe PJ et-al. Treatment of Erdheim-Chester disease with cladribine: a rational approach. Br J Ophthalmol. 2004;88 (6): 844-7. doi:10.1136/bjo.2003.035584 - Free text at pubmed - Pubmed citation
- 3. Johnson MD, Aulino JP, Jagasia M et-al. Erdheim-chester disease mimicking multiple meningiomas syndrome. AJNR Am J Neuroradiol. 2004;25 (1): 134-7. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 4. Wright RA, Hermann RC, Parisi JE. Neurological manifestations of Erdheim-Chester disease. J. Neurol. Neurosurg. Psychiatr. 1999;66 (1): 72-5. doi:10.1136/jnnp.66.1.72 - Free text at pubmed - Pubmed citation
- 5. Fortman BJ, Beall DP. Erdheim-Chester disease of the retroperitoneum: a rare cause of ureteral obstruction. AJR Am J Roentgenol. 2001;176 (5): 1330-1. AJR Am J Roentgenol (full text) - Pubmed citation
- 6. Shamburek RD, Brewer HB, Gochuico BR. Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease. Am. J. Med. Sci. 2001;321 (1): 66-75. Am. J. Med. Sci. (link) - Pubmed citation
- 7. Zanglis A, Valsamaki P, Fountos G. Erdheim-Chester disease: symmetric uptake in the (99m)Tc-MDP bone scan. Hell J Nucl Med. 11 (3): 164-7. Pubmed citation
- 8. Haaga JR, Boll D. CT and MRI of the whole body. Mosby. (2009) ISBN:0323053750. Read it at Google Books - Find it at Amazon
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling
|Include in Listings?
|Lipoid Granulomatosis
|Polyostotic Sclerosing Histiocytosis
|Non-Langerhans Cell Histiocytosis


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Nov 27 2009
The Morning Link: Hand Print Art Way more Badass Than a Turkey
Around this time each year, art teachers ask students to turn their hand print into a turkey that looks like a peacock that's been crippled by its own fat ass.
Why not let them choose what they turn their hand prints into? Sure, you might get a few idiotic variations on "The Blob with 5 Wangs," but you'd also learn which students are the right kind of idiots, which is really an art teacher's only job.
Check out these 25 badass hand print art pictures
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text: Measure the diameter of the Sun The Sun moves across the sky at a constant rate because of the rotation of the Earth. By measuring how fast the Sun moves you can work out how big the Sun appears in the sky. All you need are some household items and about 20 minutes on a sunny day.