Results 51 to 60 of about 4,926,805 (180)

Erdheim Chester disease presenting as bilateral breast dimpling and discoloration

open access: yesRadiology Case Reports
: Erdheim Chester disease is a rare disease characterized by abnormal proliferation of histiocytes. The most commonly affected site is the long bones, while involvement of the breasts is very rare. In patients with breast involvement, a unilateral breast
Christina Oska, DO, Ujas Parikh, MD
doaj   +1 more source

Bilateral Renal Colic as an Initial Presentation of Erdheim-Chester Disease

open access: yesCase Reports in Urology, 2019
Erdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis characterized by multiorgan involvement, with renal-ECD documented in over one-third of patients. Renal disease is generally asymptomatic, rarely causing hydronephrosis and kidney
Julien Sarkis   +5 more
doaj   +1 more source

Infantile Central Nervous System Juvenile Xanthogranuloma With Somatic CSF1R Mutation Responsive to Imatinib Monotherapy

open access: yesPediatric Blood &Cancer, Volume 73, Issue 10, October 2026.
ABSTRACT Juvenile xanthogranuloma (JXG) of the central nervous system (CNS) is a rare non‐Langerhans cell histiocytosis. CSF1R mutations have been reported for peripheral JXG, but not in CNS JXG. A 3‐month‐old male presented with fever, lymphadenopathy, and macrocephaly with bulging fontanelles.
Sima Vazquez   +8 more
wiley   +1 more source

Atypical skeletal involvement in patients with Erdheim–Chester disease: CT imaging findings

open access: yesOrphanet Journal of Rare Diseases, 2022
Objectives To review retrospectively atypical bone findings from computed tomographic (CT) imaging in patients with Erdheim–Chester disease. Methods All 28 patients with Erdheim–Chester disease (13 men and 15 women; mean age, 45 years; range, 7–63 years)
Zaizhu Zhang   +4 more
doaj   +1 more source

Targeted proteomics reveal histiocytosis‐associated neurodegeneration signatures

open access: yesHemaSphere, Volume 10, Issue 9, September 2026.
Abstract Neurodegeneration (ND) is a severe complication of Langerhans cell histiocytosis (LCH), yet its underlying biology and reliable biomarkers remain poorly defined. The aim of this study was to (1) gain insight into neuroimmunological mechanisms governing ND and (2) assess the clinical value of established and novel biomarkers for ND‐LCH.
Egle Kvedaraite   +29 more
wiley   +1 more source

Acute Cardiac Tamponade in a 77-year-old Italian Woman with Erdheim-Chester Disease

open access: yesEuropean Journal of Case Reports in Internal Medicine, 2016
Erdheim-Chester disease (ECD) is a non-Langerhans’ histiocytosis and a very rare multisystemic disease of unknown aetiology, with skeletal involvement of the long bones and in more than 50% of cases with extraskeletal involvement.
Maria Kyriakopoulou   +3 more
doaj   +1 more source

Pulmonary involvement in Erdheim-Chester disease

open access: yes, 2017
Erdheim–Chester disease is a disseminated non-Langerhans’ cell histiocytosis involving multiple organs with characteristic sclerotic musculoskeletal lesions.
김세규   +6 more
core   +1 more source

Rare Presentation of a Rare Orthopedic Pathology: Erdheim Chester Disease [PDF]

open access: yes, 2017
In 1930, William Chester and pathologist Jakob Erdheim discovered the pathology currently known as Erdheim Chester disease. There are only approximately 249 histologically confirmed patients to have been diagnosed with this disease as noted in the ...
Samona J, Owen J, Martin S
core   +1 more source

Histiocytosis development and clinical variation through the lens of genomics

open access: yesThe Journal of Pathology, Volume 270, Issue 1, Page 23-39, September 2026.
Abstract Histiocytic neoplasms are rare haematologic diseases characterised by clonal expansions of cells with a monocyte, macrophage or dendritic cell phenotype. Their clinical manifestations are diverse, ranging from indolent lesions to aggressive systemic disease.
Paul G Kemps   +3 more
wiley   +1 more source

Genetic analysis of primary lung interdigitating dendritic cell sarcomas

open access: yesThe Journal of Pathology, Volume 269, Issue 4-5, Page 387-398, August 2026.
Abstract Interdigitating dendritic cell sarcomas (IDCSs) are rare tumors that commonly arise in the hematopoietic system and rarely outside. The genetic drivers of IDCS carcinogenesis are unknown; therefore, therapeutic options are limited. We investigated somatic gene mutations and copy‐number alterations (CNAs) in nine IDCSs arising in the lung by ...
Mikhail S Ermakov   +6 more
wiley   +1 more source

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