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Histiocytoses, singular histiocytosis, are a rare set of conditions affecting tissue macrophages.

Historical classification of histiocytoses in a table

Features of histiocytoses:[1]

Histologic features EM features CD68 S-100 CD1a Image
Macrophage epithelioid cells, giant cells - + - - Macrophages - CD68 (WC)
Erdheim-Chester disease Touton giant cells - + +/- - ECD (upmc.edu)
Rosai-Dorfman Emperipolesis - + + - RDD (WC)
Langerhans cell histiocytosis (LCH) Reniform nuclei,
eosinophilic cytoplasm
Birbeck granules + + + LCH (WC)


The classification has evolved considerably, as the entities can overlap. An overview of a more recent classification is found in the article classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages.

Langerhans cell histiocytosis


Langerin (CD207).[2]

Erdheim-Chester disease

  • Abbreviated ECD.


  • Rare.
  • Clonal proliferation of histiocytes; 3 of 5 cases shown monoclonal by Chetritt el al.[3]



  • Bone involvement - classic.
  • Usually multiple systems are involved.




  • Foamy histocytes.
    • Large cells with gray, bubbly cytoplasm.
  • +/-Touton giant cells - very distinctive histiocytes - classic.
    • Nuclei form a ring around the cell periphery.






  • CD68 +ve.
  • S100 -ve/+ve.
  • CD1a -ve.

See also


  1. Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 479. ISBN 978-0781740517.
  2. Online 'Mendelian Inheritance in Man' (OMIM) 604862
  3. Chetritt, J.; Paradis, V.; Dargere, D.; Adle-Biassette, H.; Maurage, CA.; Mussini, JM.; Vital, A.; Wechsler, J. et al. (Sep 1999). "Chester-Erdheim disease: a neoplastic disorder.". Hum Pathol 30 (9): 1093-6. doi:10.1016/s0046-8177(99)90228-9. PMID 10492045.
  4. Sheu, SY.; Wenzel, RR.; Kersting, C.; Merten, R.; Otterbach, F.; Schmid, KW. (Nov 2004). "Erdheim-Chester disease: case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literature.". J Clin Pathol 57 (11): 1225-8. doi:10.1136/jcp.2004.018481. PMID 15509691.
  5. Haroche, J.; Amoura, Z.; Touraine, P.; Seilhean, D.; Graef, C.; Birmelé, B.; Wechsler, B.; Cluzel, P. et al. (Jun 2007). "Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review.". J Clin Endocrinol Metab 92 (6): 2007-12. doi:10.1210/jc.2006-2018. PMID 17405844.