Article
Erdheim-Chester disease.
Best practice & research. Clinical rheumatology - 1 Aug 2020
Starkebaum Gordon, Hendrie Paul
Abstract excerpt
Erdheim-Chester disease (ECD) is a rare but increasingly recognized multi-system disorder. Its diagnosis and treatment require integration of clinical information, imaging studies, and pathology studies. Of note, ECD can now be defined as a clonal myeloid disorder due to mutations which activate mitogen-activated protein kinase (MAPK) pathways and where an inflammatory milieu is important in the pathogenesis and...
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