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Search Results: 1 - 10 of 22560 matches for " erdheim-chester disease. "
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Fever of unknown origin as the first manifestation of Erdheim-Chester disease  [PDF]
Ning Li, Mingquan Chen, Huaping Sun, Yun Bao, Jiming Zhang
Case Reports in Clinical Medicine (CRCM) , 2013, DOI: 10.4236/crcm.2013.26095
Abstract: Erdheim-Chester disease (ECD) is an uncommon non-Langerhans cell histiocytosis that affects multiple body systems and can present clinically in a myriad of ways. We report the case of a 29-year-old man presented with fever and diffuse bone pain. Physical examination showed bilateral and symmetrical long bone pain, especially the knees. Radiographs showed multiple mixed bone lesions with sclerotic and lytic areas of the femora, tibiae, and fibula. Biopsy of the tibial area displayed foamy lipid-laden histiocytes, confirming the diagnosis of ECD.
Erdheim-Chester Disease with Right Atrial Tumor and “Temporal Arteritis”  [PDF]
Joseph Skalski, William D. Edwards, Eric L. Matteson
Open Journal of Rheumatology and Autoimmune Diseases (OJRA) , 2012, DOI: 10.4236/ojra.2012.23008
Abstract: Erdheim-Chester disease is an unusual syndrome characterized by non-Langerhans cell histiocytosis which can mimic rheumatologic diseases. We report a case of Erdheim-Chester masquerading as giant cell arteritis, which was success-fully managed with anakinra as anti IL-1 therapy.
Xantogranulomatosis orbitaria: Enfermedad de Erdheim-Chester Orbit Xanthogranulomatosis: Erdheim-Chester disease
P Rozas Reyes,A Se?aris González,CM González Rodríguez
Archivos de la Sociedad Espa?ola de Oftalmología , 2004,
Abstract: Caso clínico: Paciente con edema bilateral de párpados superiores y lesiones similares a xantelasmas de tres a os de evolución. La exploración oftalmológica mostraba dos tumoraciones temporales simétricas en ambos párpados superiores con lesiones naranjo-amarillentas. Se pautaron corticoides que hicieron disminuir el tama o de las tumoraciones, con recidiva de los mismos al suspender dicha medicación. La biopsia reveló una xantogranulomatosis palpebral. El resto de las exploraciones sistémicas fueron normales. Discusión: La enfermedad de Erdheim-Chester es una xantogranulomatosis que puede afectar a las estructuras oculares y periorbitarias. La combinación de lesiones similares a xantelasmas y masas orbitarias bilaterales deben hacernos pensar en este proceso y buscar la posible afectación sistémica. Case report: A patient was studied because of upper lid bilateral edema and xanthelasmae-like lesions after three years of evolution. During the ophthalmologic examination orange-yellowish lesions and two symmetrical tumours were observed on the temporal part of both upper lids. Corticoid-therapy was undertaken which reduced the size of the tumours, however the size increased again after the discontinuation of treatment. A biopsy was performed and lid xanthogranulomatosis was diagnosed. Other systemic examinations were normal. Discussion: Erdheim-Chester disease is a xanthogranulomatosis that can affect ocular and periorbital structures. Combination of xanthelasmae-like lesions and bilateral orbital masses should make us consider this process and try to locate any associated systemic conditions.
Xantogranulomatosis orbitaria: Enfermedad de Erdheim-Chester
Rozas Reyes,P; Se?aris González,A; González Rodríguez,CM;
Archivos de la Sociedad Espa?ola de Oftalmología , 2004, DOI: 10.4321/S0365-66912004001000009
Abstract: case report: a patient was studied because of upper lid bilateral edema and xanthelasmae-like lesions after three years of evolution. during the ophthalmologic examination orange-yellowish lesions and two symmetrical tumours were observed on the temporal part of both upper lids. corticoid-therapy was undertaken which reduced the size of the tumours, however the size increased again after the discontinuation of treatment. a biopsy was performed and lid xanthogranulomatosis was diagnosed. other systemic examinations were normal. discussion: erdheim-chester disease is a xanthogranulomatosis that can affect ocular and periorbital structures. combination of xanthelasmae-like lesions and bilateral orbital masses should make us consider this process and try to locate any associated systemic conditions.
Doen?a de Erdheim-Chester: relato de dois casos
Hexsel, Fernando Fernandez;Canazaro, Letícia Frigo;Capoani, Mariana;Aguiar, Paula Musa;Suwa, Eiji;Maciel, Antonio Carlos;
Radiologia Brasileira , 2009, DOI: 10.1590/S0100-39842009000400015
Abstract: erdheim-chester disease is a rare non-langerhans cell histiocytosis of unknown etiology, affecting multiple organ system, involving bones, central nervous system, eyes, lungs, mediastinum, kidneys and retroperitoneum. the authors report two cases that progressed with the typical presentation of the disease. radiological findings were in agreement with literature and guided the diagnosis, confirmed by immunohistochemistry.
Enfermedad de Erdheim-Chester e histiocitosis de células de Langerhans: ?Una asociación fortuita?
Simiele Narvarte,A.; Novoa Sanjurjo,F.; Gómez Rodríguez,N.; Antón Badiola,I.;
Anales de Medicina Interna , 2004, DOI: 10.4321/S0212-71992004001200006
Abstract: erdheim-chester disease is an idiopathic systemic histiocytosis hystologically different to langerhans histiocytosis which is characterized by symmetric sclerotic bone lesions, predominantly affecting the diaphyseal and metaphyseal areas of large bones. it is not classified within malignant histicytosis, but the clinical course tends to be agressive with a poor reponse to different schedules of treatment. in rare cases the association of both entities has been reported: erdheim-chester disease and langerhans histiocytosis. we present a new case with histological data of both histiocytosis whose clinical course included bone and muscle pain, insipidus diabetes, exophthalmos, bilateral symmetrical sclerosing bone lesions and a cerebellar syndrome.
Enfermedad de Erdheim-Chester. Una causa rara de derrame pericárdico: Caso clínico
Vega,Jorge; Cisternas,Marcela; Bergoeing,Michel; Espinosa,Roberto; Zapico,Alvaro; Chadid,Pedro; Santamarina,Mario;
Revista médica de Chile , 2011, DOI: 10.4067/S0034-98872011000800011
Abstract: we report a 76-year-old male who was admitted due to progressive congestive heart failure lasting several months. an echocardiogram showed a large pericardial effusion with early signs of pericardial tamponade and an irregular surface suggestive of cancer infiltration. the patient was operated, creating a pericardial window and draining 1,200 ml of a brownish yellow fluid with abundant cellularity. pericardial biopsy showed infiltration by cd68 (+), cd1a (-) and s100 (-) cells. twenty-eight months earlier, due to fatigue, dyspnea, and a non-specific inflammatory process, an enhanced-contrast-scan showed that aorta was coated with a hypodense tissue that began near the aortic valve and extended until the inferior mesenteric artery, with stenosis of the left subclavian, celiac axis, renal and upper mesenteric arteries. an angioplasty and stent placing was carried out in the last two arteries. both kidneys had the appearance of "hairy kidneys". a bone scan showed increased uptake in femurs and tibiae and x-ray examination showed osteosclerosis in metaphysis and diaphysis. the diagnosis of erdheim-chester disease (non-langerhans-cell histiocytosis) was made and the patient was treated with steroids and methotrexate.
November 2012 imaging case of the month
Gotway MB
Southwest Journal of Pulmonary and Critical Care , 2012,
Abstract: No abstract available. Article truncated at end of first page. Clinical History: A 61-year-old non-smoking man presented with a history of dyspnea on exertion; fatigue; and worsening lower extremity edema. Frontal chest radiography (Figure 1) was performed.Figure 1. Frontal chest radiographyWhich of the following statements regarding the chest radiograph is most accurate?1.The frontal chest radiograph is normal 2.The frontal chest radiograph shows a diffuse linear; interstitial pattern3.The frontal chest radiograph shows multilobar consolidation4.The frontal chest radiograph shows numerous thin-walled cysts5.The frontal chest radiograph shows numerous small nodules consistent with a “miliary” pattern…
Enfermedad de Erdheim-Chester. Una causa rara de derrame pericárdico: Caso clínico Erdhei-Chester disease: Report of one case
Jorge Vega,Marcela Cisternas,Michel Bergoeing,Roberto Espinosa
Revista médica de Chile , 2011,
Abstract: We report a 76-year-old male who was admitted due to progressive congestive heart failure lasting several months. An echocardiogram showed a large pericardial effusion with early signs of pericardial tamponade and an irregular surface suggestive of cancer infiltration. The patient was operated, creating a pericardial window and draining 1,200 ml of a brownish yellow fluid with abundant cellularity. Pericardial biopsy showed infiltration by CD68 (+), CD1a (-) and S100 (-) cells. Twenty-eight months earlier, due to fatigue, dyspnea, and a non-specific inflammatory process, an enhanced-contrast-scan showed that aorta was coated with a hypodense tissue that began near the aortic valve and extended until the inferior mesenteric artery, with stenosis of the left subclavian, celiac axis, renal and upper mesenteric arteries. An angioplasty and stent placing was carried out in the last two arteries. Both kidneys had the appearance of "hairy kidneys". A bone scan showed increased uptake in femurs and tibiae and X-ray examination showed osteosclerosis in metaphysis and diaphysis. The diagnosis of Erdheim-Chester disease (non-Langerhans-cell histiocytosis) was made and the patient was treated with steroids and methotrexate.
Acometimento intra-ocular na doen?a de Erdheim-Chester - primeiro relato na literatura: relato de caso
Biccas Neto, Laurentino;Zanetti, Fernando;
Arquivos Brasileiros de Oftalmologia , 2007, DOI: 10.1590/S0004-27492007000500025
Abstract: erdheim-chester disease (ecd) is a granulomatous and infiltrative disorder of unknown etiology with proliferation of cholesterol-containing histiocytes and peculiar bone involvement. it is very similar to langerhans cell histiocytosis (lch) on histology but with a different immunohistochemical profile. this is the first report of intraocular involvement in this disease. mpsg, a 46 y.o. woman, presented with proptosis of the od. she referred ulcerated lesions on the hard palate, symmetrical and bilateral osteosclerosis of the fibulae and tibiae and a nodule in the right breast (biopsy: xantomatous histiocytic infiltrate cd68+, s-100 and cd1a negative on immunohistochemistry compatible with ecd). mri studies demonstrated an extraconal tumor in the juxta-bulbar temporal portion of the right orbit close to the lacrimal gland and hyperintense on t1. vision was 20/20 ou, with numerous drusen in the posterior pole, similar to basal laminar drusen. two regions of orange subretinal infiltrates that showed progressive staining on the angiogram were seen in the peripapillary region and also close to the fovea in the od. choroidal neovascular membranes were seen 2 years later in ou leading to severe visual loss in the os and to a slight visual field loss in the od, which retained 20/20 vision. this pioneer report depicts in vivo characteristics of histiocytic granulomas in ecd. caution should be taken with patients with ecd as potentially blinding intraocular complications may arise.
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