Erdheim–Chester disease

Erdheim–Chester disease is a rare non-Langerhans cell histiocytic disorder of unknown cause characterized by heterogeneous systemic manifestation that usually affects adults. The definition of a histiocyte has expanded over time form it original conception as a macrophages to now include both macrophagic and monocytic cells involved in phagocytosis, antigen presentation, and other functions in the immune response.

It was first described as ‘lipid granulomatose’ by Jacob Erdheim’s student William Chester in 1930.

It is microscopically characterized by lipid-laden foamy macrophages, chronic inflammatory cells, and varying degrees of fibrosis.

Extraskeletal manifestations are present in around one half of cases, and the sites of involvement also include the skin, orbit, hypothalamus/pituitary and retroperitoneum: these sites may be more often involved than the lung.

Erdheim–Chester disease is believed to be non-inherited disorder of middle-aged patients with a slight male preponderance. Report published in 2012, there is being a strong male predominance in this disease, with 73% of patients being male and only 27% female.
Erdheim–Chester disease