Other Names for this Disease
- Erdheim Chester disease
- Lipoid granulomatosis
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 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. As a result, these tissues and organs become thickened, dense and fibrotic. 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. Without successful treatment, organ failure can occur.Erdheim-Chester disease is a rare condition that can affect many different organs of the body.
Last updated: 3/2/2010
- Shamburek RD. Erdheim Chester Disease. National Organization for Rare Disorders (NORD). 2009; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Erdheim%20Chester%20Disease. Accessed 3/2/2010.
- What is Erdheim-Chester Disease. ECD Global Alliance. 2009; http://www.erdheim-chester.org/. Accessed 3/2/2010.
- The Doctor's Doctor web site provides information about Erdheim-Chester disease. Click on the link above to access this information.
- Genetics Home Reference (GHR) contains information on Erdheim-Chester disease. This website is maintained by the National Library of Medicine.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- 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.