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We report a 67\u2010year\u2010old man who had fatal multi\u2010systemic Erdheim\u2013Chester disease involvement, including retroperitoneal fibrosis, dilated cardiomyopathy and bone pain that was diagnosed late in its course and was refractory to interferon\u2010alfa. A pathogenic <jats:styled-content style=\"fixed-case\">BRAF<jats:sup>V600E<\/jats:sup><\/jats:styled-content> mutation, identified after death in this patient, provides insights into pathogenesis and opens potential lines of treatment.<\/jats:p>","DOI":"10.1111\/imj.12685","type":"journal-article","created":{"date-parts":[[2015,3,3]],"date-time":"2015-03-03T10:49:33Z","timestamp":1425379773000},"page":"348-351","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Does the <scp>BRAF<sup>V600E<\/sup><\/scp> mutation herald a new treatment era for <scp>E<\/scp>rdheim\u2013<scp>C<\/scp>hester disease? A case\u2010based review of a rare and difficult to diagnose disorder"],"prefix":"10.1111","volume":"45","author":[{"given":"J.","family":"Bosco","sequence":"first","affiliation":[{"name":"Department of Clinical Immunology and Allergy Westmead Hospital  Sydney New South Wales Australia"},{"name":"Department of Allergy Immunology and Respiratory Medicine Alfred Hospital  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Allende","sequence":"additional","affiliation":[{"name":"Department of Tissue Pathology and Diagnostic Oncology Westmead Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W.","family":"Varikatt","sequence":"additional","affiliation":[{"name":"Department of Tissue Pathology and Diagnostic Oncology Westmead Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Radiology Westmead Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. 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