{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:15:42Z","timestamp":1787026542149,"version":"build-2736575974"},"reference-count":20,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,7,14]],"date-time":"2025-07-14T00:00:00Z","timestamp":1752451200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Erdheim-Chester disease (ECD) is an exceedingly rare non-Langerhans histiocytosis. While often affecting the skeleton, cardiovascular system, and kidneys, pancreatic involvement remains uncommon and can mimic more prevalent conditions such as autoimmune or chronic pancreatitis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>\n                      A 58-year-old female presented with a two-year history of bilateral lower limb edema and a year-long course of recurrent abdominal pain. Imaging suggested necrotizing pancreatitis and retroperitoneal infiltration, yet serum IgG4 levels were normal. A CT-guided biopsy of the pancreas and retroperitoneum revealed diffuse proliferation of foamy histiocytes (CD68\n                      <jats:sup>+<\/jats:sup>\n                      , CD163\n                      <jats:sup>+<\/jats:sup>\n                      , CD1\u03b1\n                      <jats:sup>-<\/jats:sup>\n                      ) carrying the BRAF V600E mutation, confirming ECD. Supportive therapy and corticosteroids temporarily relieved symptoms, but targeted treatment was delayed due to the COVID-19 pandemic. Subsequent follow-up revealed significant clinical improvement following targeted therapy.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>ECD can present with non-specific clinical features, leading to frequent misdiagnoses. Involvement of the pancreas, as demonstrated here, is particularly rare. The discovery of the BRAF V600E mutation underscores the importance of molecular testing for both diagnostic confirmation and therapeutic stratification. The immunopathogenesis of ECD involves activated macrophages and aberrant MAPK signaling, which drive chronic inflammation and tissue fibrosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights the diagnostic challenges of pancreatic ECD and underscores the critical value of an integrated approach\u2014including imaging, immunohistochemistry, and molecular analysis\u2014in achieving timely diagnosis. Early recognition and targeted therapy may significantly improve outcomes for patients with BRAF-mutant ECD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2025.1611452","type":"journal-article","created":{"date-parts":[[2025,7,14]],"date-time":"2025-07-14T04:10:29Z","timestamp":1752466229000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case Report: The rare pancreatic involvement in Erdheim-Chester disease"],"prefix":"10.3389","volume":"16","author":[{"given":"Ji","family":"Li","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yadan","family":"Zou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juanjuan","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ting","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheng-Guang","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,7,14]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"561","DOI":"10.1007\/BF01942684","article-title":"\u00dcber lipoidgranulomatose","volume":"279","author":"Chester","year":"1930","journal-title":"Virchows Arch Pathol Anat"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000145368.17934.91","article-title":"Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review","volume":"83","author":"Haroche","year":"2004","journal-title":"Med (Baltimore)"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-05-430140","article-title":"High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses","volume":"120","author":"Haroche","year":"2012","journal-title":"Blood"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.5114\/pjr.2018.73290","article-title":"Imaging findings in Erdheim-Chester disease: what every radiologist needs to know","volume":"83","author":"Kumar","year":"2018","journal-title":"Pol J Radiol"},{"key":"B5","doi-asserted-by":"publisher","first-page":"e6996","DOI":"10.1002\/ccr3.6996","article-title":"Erdheim-Chester disease: a multisystem disease case illustration with rare manifestations and treatment challenges","volume":"11","author":"Jridi","year":"2023","journal-title":"Clin Case Rep"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1016\/j.radcr.2023.02.012","article-title":"Pancreatic involvement in Erdheim-Chester disease: Rare presentation of a rare disease","volume":"18","author":"Rafiee","year":"2023","journal-title":"Radiol Case Rep"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1097\/00003086-198405000-00039","article-title":"Erdheim-Chester disease. 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