Multisystem Radiologic Manifestations of Erdheim-Chester Disease

Erdheim-Chester Disease is a rare form of multiorgan non-Langerhans’ cell histiocytosis that affects individuals between the ages of 50 and 70 with an equal distribution among males and females. It is associated with significant morbidity and mortality that is mostly due to infiltration of critical...

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Main Authors: Umairullah Lodhi, Uzair Sarmast, Saadullah Khan, Kavitha Yaddanapudi
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Radiology
Online Access:http://dx.doi.org/10.1155/2016/2670495
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author Umairullah Lodhi
Uzair Sarmast
Saadullah Khan
Kavitha Yaddanapudi
author_facet Umairullah Lodhi
Uzair Sarmast
Saadullah Khan
Kavitha Yaddanapudi
author_sort Umairullah Lodhi
collection DOAJ
description Erdheim-Chester Disease is a rare form of multiorgan non-Langerhans’ cell histiocytosis that affects individuals between the ages of 50 and 70 with an equal distribution among males and females. It is associated with significant morbidity and mortality that is mostly due to infiltration of critical organs. Some of the sites that Erdheim-Chester Disease affects include the skeletal system, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum), and skin. The most common presenting symptom of Erdheim-Chester Disease is bone pain although a large majority of patients are diagnosed incidentally during a workup for a different disease process. Diagnosing Erdheim-Chester Disease is challenging due its rarity and mimicry to other infiltrative processes. Therefore, a multimodality diagnostic approach is employed with imaging being at the forefront. As of date, a comprehensive radiologic review of the manifestations of Erdheim-Chester Disease has rarely been reported. Here we present radiologic findings of an individual suffering from Erdheim-Chester Disease.
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spelling doaj-art-0b37f5d971d840178913399fac4edfcd2025-08-20T02:03:43ZengWileyCase Reports in Radiology2090-68622090-68702016-01-01201610.1155/2016/26704952670495Multisystem Radiologic Manifestations of Erdheim-Chester DiseaseUmairullah Lodhi0Uzair Sarmast1Saadullah Khan2Kavitha Yaddanapudi3Department of Radiology, Stony Brook University Hospital, 101 Nicolls Road, Stony Brook, NY 11794, USADepartment of Radiology, Stony Brook University Hospital, 101 Nicolls Road, Stony Brook, NY 11794, USADepartment of Radiology, Stony Brook University Hospital, 101 Nicolls Road, Stony Brook, NY 11794, USADepartment of Radiology, Stony Brook University Hospital, 101 Nicolls Road, Stony Brook, NY 11794, USAErdheim-Chester Disease is a rare form of multiorgan non-Langerhans’ cell histiocytosis that affects individuals between the ages of 50 and 70 with an equal distribution among males and females. It is associated with significant morbidity and mortality that is mostly due to infiltration of critical organs. Some of the sites that Erdheim-Chester Disease affects include the skeletal system, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum), and skin. The most common presenting symptom of Erdheim-Chester Disease is bone pain although a large majority of patients are diagnosed incidentally during a workup for a different disease process. Diagnosing Erdheim-Chester Disease is challenging due its rarity and mimicry to other infiltrative processes. Therefore, a multimodality diagnostic approach is employed with imaging being at the forefront. As of date, a comprehensive radiologic review of the manifestations of Erdheim-Chester Disease has rarely been reported. Here we present radiologic findings of an individual suffering from Erdheim-Chester Disease.http://dx.doi.org/10.1155/2016/2670495
spellingShingle Umairullah Lodhi
Uzair Sarmast
Saadullah Khan
Kavitha Yaddanapudi
Multisystem Radiologic Manifestations of Erdheim-Chester Disease
Case Reports in Radiology
title Multisystem Radiologic Manifestations of Erdheim-Chester Disease
title_full Multisystem Radiologic Manifestations of Erdheim-Chester Disease
title_fullStr Multisystem Radiologic Manifestations of Erdheim-Chester Disease
title_full_unstemmed Multisystem Radiologic Manifestations of Erdheim-Chester Disease
title_short Multisystem Radiologic Manifestations of Erdheim-Chester Disease
title_sort multisystem radiologic manifestations of erdheim chester disease
url http://dx.doi.org/10.1155/2016/2670495
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