Erdheim-Chester disease: Difference between revisions

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{{CMG}}
{{CMG}}


{{SK}} Erdheim-Chester syndrome; polyostotic sclerosing histiocytosis
{{SK}} Erdheim-Chester syndrome; polyostotic sclerosing histiocytosis; ECD


==Overview==
==[[Erdheim-Chester disease overview|Overview]]==


==History==
==[[Erdheim-Chester disease historical perspective|Historical Perspective]]==


==Clinical Presentation==
==[[Erdheim-Chester disease pathophysiology|Pathophysiology]]==


==Histology==
==[[Erdheim-Chester disease causes|Causes]]==
Histologically, ECD differs from Langerhans cell histiocytosis (LCH) in a number of ways. Unlike LCH, ECD does not stain positive for [[S-100 protein|S-100]] or [[CD 1a]], and electron microscopy of cell cytoplasm does not disclose [[Birbeck granules]].<ref name="pmid8965684"> </ref>  Tissue samples show xanthomatous or xanthogranulomatous infiltration by lipid-laden or foamy histiocytes, and are usually surrounded by fibrosis. Bone biopsy is said to offer the greatest likelihood of reaching a diagnosis.
 
==[[Erdheim-Chester disease differential diagnosis|Differentiating Erdheim-Chester disease from other Diseases]]==
 
==[[Erdheim-Chester disease epidemiology and demographics|Epidemiology and Demographics]]==
 
==[[Erdheim-Chester disease natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==Diagnosis==
==Diagnosis==
Radiologic osteosclerosis and histology are the main diagnostic features. Diagnosis can often be difficult because of the rareness of ECD as well as the need to differentiate it from LCH. A diagnosis from neurological imaging may not be definitive. The presence of symmetrical cerebellar and pontine signal changes on T2-weighted images seem to be typical of ECD, however, multiple sclerosis and metabolic diseases must also be considered in the differential diagnosis.<ref>{{cite journal  | last = Weidauer  | first =S.  | coauthors =von Stuckrad-Barre S., Dettmann E., Zanella F.E., & Lanfermann H.  | title =Cerebral Erdheim-Chester disease: case report and review of the literature.  | journal = Neuroradiology | volume =45 | pages =241-245  | date =2002}}</ref>
[[Erdheim-Chester disease history and symptoms|History and Symptoms]] | [[Erdheim-Chester disease physical examination|Physical Examination]] | [[Erdheim-Chester disease laboratory findings|Laboratory Findings]] [[Erdheim-Chester disease electrocardiogram|Electrocardiogram]] | [[Erdheim-Chester disease x ray|X Ray]] | [[Erdheim-Chester disease CT|CT]] | [[Erdheim-Chester disease MRI|MRI]] | [[Erdheim-Chester disease echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Erdheim-Chester disease other imaging findings|Other Imaging Findings]] | [[Erdheim-Chester disease other diagnostic studies|Other Diagnostic Studies]]
 
ECD is not a common cause of exophthalmos but can be diagnosed by biopsy.  However, like all biopsies, this may be inconclusive.<ref name="Mdanderson"> {{cite web |url=http://www.mdanderson.org/diseases/eyecancer/display.cfm?id=5423a04c-459e-4e12-a737309ef2e1e299&method=displayfull |title=Erdheim Chester Disease - M. D. Anderson Cancer Center |accessdate=2007-08-26 |format= |work=}}</ref>


==Treatment==
==Treatment==
[[Erdheim-Chester disease medical therapy|Medical Therapy]] | [[Erdheim-Chester disease surgery|Surgery]] | [[Erdheim-Chester disease cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Erdheim-Chester disease future or investigational therapies|Future or Investigational Therapies]]
<!-- Use the following categories
==Prognosis==
==Further Reading==
==External Links==
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==References==
==Case Studies==
{{Reflist|2}}
[[Erdheim-Chester disease case study one|Case #1]]


{{Hematology}}
{{Hematology}}

Latest revision as of 19:36, 21 September 2012

Erdheim-Chester disease
ICD-10 C96.1
ICD-9 202.3
DiseasesDB 29792
MeSH D031249

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Erdheim-Chester syndrome; polyostotic sclerosing histiocytosis; ECD

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Erdheim-Chester disease from other Diseases

Epidemiology and Demographics

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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