A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis

Erdheim-Chester disease (ECD) is an exceedingly rare and aggressive disease characterized by foamy CD68 + CDa-histiocytic infiltration into multiple tissues and organs. Only 1,500 cases have been diagnosed since 1930 when ECD was first described. Biliary tract involvement of ECD has only been report...

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Autores principales: Kamalumpundi, Vijayvardhan, Rieth, John, Tran, Huy, Ashat, Munish, Zhang, Xiaocen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer 2023
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9857465/
https://www.ncbi.nlm.nih.gov/pubmed/36699184
http://dx.doi.org/10.14309/crj.0000000000000963
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author Kamalumpundi, Vijayvardhan
Rieth, John
Tran, Huy
Ashat, Munish
Zhang, Xiaocen
author_facet Kamalumpundi, Vijayvardhan
Rieth, John
Tran, Huy
Ashat, Munish
Zhang, Xiaocen
author_sort Kamalumpundi, Vijayvardhan
collection PubMed
description Erdheim-Chester disease (ECD) is an exceedingly rare and aggressive disease characterized by foamy CD68 + CDa-histiocytic infiltration into multiple tissues and organs. Only 1,500 cases have been diagnosed since 1930 when ECD was first described. Biliary tract involvement of ECD has only been reported in the literature once. We report a case of ECD causing extrahepatic biliary obstruction without significant bile duct dilation, mimicking primary sclerosing cholangitis or IgG4 disease.
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spelling pubmed-98574652023-01-24 A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis Kamalumpundi, Vijayvardhan Rieth, John Tran, Huy Ashat, Munish Zhang, Xiaocen ACG Case Rep J Case Report Erdheim-Chester disease (ECD) is an exceedingly rare and aggressive disease characterized by foamy CD68 + CDa-histiocytic infiltration into multiple tissues and organs. Only 1,500 cases have been diagnosed since 1930 when ECD was first described. Biliary tract involvement of ECD has only been reported in the literature once. We report a case of ECD causing extrahepatic biliary obstruction without significant bile duct dilation, mimicking primary sclerosing cholangitis or IgG4 disease. Wolters Kluwer 2023-01-20 /pmc/articles/PMC9857465/ /pubmed/36699184 http://dx.doi.org/10.14309/crj.0000000000000963 Text en © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Case Report
Kamalumpundi, Vijayvardhan
Rieth, John
Tran, Huy
Ashat, Munish
Zhang, Xiaocen
A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis
title A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis
title_full A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis
title_fullStr A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis
title_full_unstemmed A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis
title_short A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis
title_sort case of erdheim-chester disease causing secondary sclerosing cholangitis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9857465/
https://www.ncbi.nlm.nih.gov/pubmed/36699184
http://dx.doi.org/10.14309/crj.0000000000000963
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