Document Detail

Erdheim-Chester disease. Case report with autopsy findings.
MedLine Citation:
PMID:  2039348     Owner:  NLM     Status:  MEDLINE    
Erdheim-Chester disease is a rare pathologic entity characterized by symmetrical radiodensities in the metaphyseal and the diaphyseal portions of the long bones. Fibrosis, osteoblastic cortical bone deposition, and fibroxanthomatous granulomas with lipid-laden macrophages and multinucleated giant cells, which have a particular tropism for connective and adipose tissues, are the pathologic hallmarks. To our knowledge, 27 cases have been reported in the literature since the entity was first described in 1930. Protean clinical features range from a focal and asymptomatic process to a multisystemic infiltrative disease. We describe the clinical course of a new case and review the extensive pathologic findings at autopsy, including those demonstrated by light and electron microscopy and cytochemical and immunocytochemical studies.
M G Fink; D J Levinson; N L Brown; S Sreekanth; G W Sobel
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Archives of pathology & laboratory medicine     Volume:  115     ISSN:  0003-9985     ISO Abbreviation:  Arch. Pathol. Lab. Med.     Publication Date:  1991 Jun 
Date Detail:
Created Date:  1991-06-28     Completed Date:  1991-06-28     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7607091     Medline TA:  Arch Pathol Lab Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  619-23     Citation Subset:  AIM; IM    
Department of Pathology, Humana Hospital-Michael Reese, Chicago, Ill. 60616.
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MeSH Terms
Adipose Tissue / pathology
Bone Diseases / complications,  pathology*
Bone and Bones / pathology
Connective Tissue / pathology
Diabetes Insipidus / complications
Diabetes Mellitus, Type 1 / complications
Heart Failure / complications
Immunoenzyme Techniques
Kidney Diseases / complications,  pathology
Lipid Metabolism
Macrophages / pathology
Myocardial Infarction / complications
Testis / pathology
Xanthomatosis / complications

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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