Lymph nodes - not lymphoma
Neoplasms (not lymphoma)
Erdheim-Chester disease

Topic Completed: 1 May 2014

Revised: 5 February 2019

Copyright: (c) 2003-2018,, Inc.

PubMed Search: Erdheim-Chester disease[TI] free full text[sb]

See also: Bone chapter

Patricia Tsang, M.D., M.B.A.
Page views in 2018: 1,358
Page views in 2019 to date: 1,172
Cite this page: Tsang P. Erdheim-Chester disease. website. Accessed August 21st, 2019.
Definition / general
  • Rare non-Langerhans cell histiocytosis of bone of unknown etiology, with 50% also involving hypothalamic pituitary axis, lung, heart, retroperitoneum, skin, liver, kidneys, spleen, orbit
  • Nodal involvement is very rare, despite multisystem disease
Clinical features
  • Most common symptom is bone pain, usually involving lower extremities
  • Broad extraskeletal manifestations, including fever, weight loss, weakness, exophthalmos, diabetes insipidus, dysuria, abdominal pain, obstructive renal impairment, ataxia and paresis
  • Interstitial lung disease in 35% of patients with histiocytic cells and fibrosis seen in a predominantly perilymphangitic and subpleural pattern
  • Prognosis depends on extent and distribution of extraskleletal manifestations
  • Most common causes of death are respiratory distress, extensive pulmonary fibrosis and cardiac failure
Radiology description
  • Pathognomonic radiography showing bilateral, symmetrical osteosclerosis of metaphyseal and diaphyseal regions and sparing of the epiphyses
Radiology images

Images hosted on PathOut server:

Pathological fracture of left tibia

Case reports
Microscopic (histologic) description
  • Large foamy histiocytes containing lipid, lymphocytic aggregates, fibrosis
  • Rare Touton-like giant cells
Microscopic (histologic) images

Images hosted on PathOut server:

Neck lymph node biopsy

Skin lesion biopsy


Images hosted on other servers:


Testis, thyroid, lymph node - CD68

CNS tumor is CD68+ with rare S100+ cells

CD68, factor XIIIa, CD163

Cytology images

Images hosted on other servers:

Histiocytes from touch preparation

Positive stains
Negative stains
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