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Genetic and Rare Diseases Information Center (GARD)

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Erdheim-Chester disease

Other Names for this Disease
  • ECD
  • Erdheim Chester disease
  • Lipoid granulomatosis
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Erdheim-Chester disease is a rare condition that can affect many different organs of the body.[1][2] This condition, which usually affects adults, is characterized by excessive production and accumulation of histiocytes (specific cells that normally play a role in responding to infection and injury) within multiple tissues and organs.[1] As a result, these tissues and organs become thickened, dense and fibrotic.[2] Sites of involvement may include the long bones, skin, tissues behind the eyeballs, lungs, brain, and pituitary gland, among others. Signs and symptoms, as well as disease course, depend on the specific location and extent of involvement.[1] Without successful treatment, organ failure can occur.[2]
Last updated: 3/2/2010


  1. Shamburek RD. Erdheim Chester Disease. National Organization for Rare Disorders (NORD). 2009; Accessed 3/2/2010.
  2. What is Erdheim-Chester Disease. ECD Global Alliance. 2009; Accessed 3/2/2010.
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Basic Information

  • The Doctor's Doctor web site provides information about Erdheim-Chester disease. Click on the link above to access this information.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Erdheim-Chester disease. Click on the link to view a sample search on this topic.

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