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Concept information

Hemic and Lymphatic Diseases > Lymphatic Diseases > Histiocytosis > Histiocytosis, Non-Langerhans-Cell > Erdheim-Chester Disease

Preferred term

Erdheim-Chester Disease  

Type

  • Topical Descriptor

Broader concept

Entry terms

  • Granulomatosis, Lipid
  • Lipid Granulomatosis

Scope note

  • A rare form of non-Langerhans-cell histiocytosis (HISTIOCYTOSIS, NON-LANGERHANS-CELL) with onset in middle age. The systemic disease is characterized by infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.

History note

  • 2003

In other languages

URI

http://www.yso.fi/onto/mesh/D031249

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