Disease Information for Erdheim Chester Disease

Erdheim-Chester disease

Also known as:

Lipogranulomatosus Erdheim-Chester

Synonyms:

Lipid granulomatosis.

Associated persons:

William Chester

Jakob Erdheim

Description:

A rare histiocytic disorder characterised by lipoid granulomas in various parts of the body and in various organs. Clinical manifestations range from asymptomatic to fatal multisystem involvement, typically bones, kidneys, heart, and lungs.

First described by Chester in 1931. The eponym Erdheim-Chester was coined later, despite the fact that Chester, who then worked in the pathological institute in Vienna, was sole author.

[whonamedit.com 2005]

---------------------------------------------------------------------Erdheim Chester Disease

ECD

Lipoid Granulomatosis

Disorder Subdivisions

General Discussion

Erdheim-Chester disease (ECD) is a rare multisystem disorder of adulthood. It is characterized by excessive production and accumulation of histiocytes within multiple tissues and organs. Histiocytes are large phagocytic cells (macrophages) that normally play a role in responding to infection and injury. (A phagocytic cell is any "scavenger cell" that engulfs and destroys invading microorganisms or cellular debris.) In those with ECD, sites of involvement may include the long bones, skin, tissues behind the eyeballs, lungs, brain, pituitary gland, and/or additional tissues and organs. Associated symptoms and findings and disease course depend on the specific location and extent of such involvement. The specific underlying cause of ECD is unknown.

[NORD 2005}

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