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Article

Erdheim-Chester disease.

Current rheumatology reports - 1 Apr 2014

Haroche Julien, Arnaud Laurent, Cohen-Aubart Fleur, Hervier Baptiste, Charlotte Frédéric, Emile Jean-François, Amoura Zahir

Abstract excerpt

Erdheim-Chester disease (ECD) is a rare (approximately 500 known cases worldwide), non-inherited, non-Langerhans form of histiocytosis of unknown origin, first described in 1930. It is characterized by xanthomatous or xanthogranulomatous infiltration of tissues by foamy histiocytes, "lipid-laden" macrophages, or histiocytes, surrounded by fibrosis. Diagnosis of ECD involves the analysis of histiocytes in tissue...

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Source

Identifiers

PMID: 24532298
DOI: 10.1007/s11926-014-0412-0

Genes

ECD
BRAF

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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