ArticleVirchows Archiv : an international journal of pathology2008
Systemic Erdheim-Chester disease.
Article in Virchows Archiv : an international journal of pathology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 41 citations in OpenAlex.
- Erdheim-Chester Disease with Pancreatic Enlargement Observed Using Contrast-enhanced Endoscopic Ultrasonography.Internal medicine (Tokyo, Japan) · 2025Article
- Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review.BMC musculoskeletal disorders · 2021Review
- Primary Intraosseous Xanthomas of the Jaws: A Series of Six Cases Including an Example with Formation of Apoptosis-Related Hyaline Globules, So-Called "Thanatosomes".Head and neck pathology · 2020Article
- The First Reported Case of Erdheim-Chester Disease in Egypt with Bilateral Exophthalmos, Loss of Vision, and Multi-Organ Involvement in a Young Woman.The American journal of case reports · 2016Article
- [Erdheim-Chester disease and Rosai-Dorfman disease: Pathological, radiological and clinical features of adult non-Langerhans cell histiocytosis].Der Pathologe · 2015Review
- Erdheim-Chester disease: from palliative care to targeted treatment.Clinical kidney journal · 2014Article
- Bilateral orbital Erdheim-Chester disease treated with 12 weekly administrations of VNCOP-B chemotherapy: a case report and a review of literature.Rheumatology international · 2012Review
- FDG-PET in the Erdheim-Chester disease: its diagnostic and follow-up role.Rheumatology international · 2012Article
- Erdheim-Chester disease presenting with cutaneous involvement: a case report and literature review.Journal of cutaneous pathology · 2011Review
- Erdheim-chester disease associated with marginal zone lymphoma and monoclonal proteinemia.Case reports in hematology · 2011Article
- Erdheim-chester disease.Annals of dermatology · 2010Article
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Erdheim-Chester disease is a rare xanthomatosis that may present with characteristic radiologic and histologic features. There have been conflicting reports regarding the nature of this process, including whether it represents a reactive or neoplastic lesion. We present the clinical histories, pathologic findings, and an analysis of clonality using the HUMARA assay in two patients diagnosed with Erdheim-Chester disease. One case has previously been documented in the literature. Histologically, both cases demonstrated sheets of foamy xanthomatous histiocytes with widespread infiltration of the viscera. These regions were punctuated by variable amounts of inflammation, including lymphocytes, plasma cells, and occasional Touton-type giant cells. The histiocytes were immunoreactive for CD68 and CD163; they did not stain with S100 or CD1a. One case was found to be monoclonal; however, the second case had extensive DNA degradation; thus, clonality could not be assessed. In addition to contributing an additional report of this rare disease to the literature, we demonstrate the histiocytes to express CD163, thereby further supporting a monocyte/macrophage basis. Moreover, in confirming clonality, our observations lend additional evidence to the view that Erdheim-Chester disease represents a neoplastic process.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.