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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.
Erdheim-Chester Disease Associated with Marginal Zone Lymphoma and Monoclonal Proteinemia
Peter G. Pavlidakey,Alok Mohanty,Lisa J. Kohler,Howard J. Meyerson
Case Reports in Hematology , 2011, DOI: 10.1155/2011/941637
Abstract: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. We report a fatal case of ECD with extensive cardiac involvement associated with a marginal zone lymphoma and monoclonal proteinemia in a young man. This is the first reported association of ECD with a monoclonal gammopathy or a lymphoma.
Xantogranulomatosis orbitaria: Enfermedad de Erdheim-Chester Orbit Xanthogranulomatosis: Erdheim-Chester disease  [cached]
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.
Bilateral orbital involvement in Erdheim-Chester disease: case report
Monteiro, Mário Luiz Ribeiro;Sampaio, César Moreira;
Arquivos Brasileiros de Oftalmologia , 2002, DOI: 10.1590/S0004-27492002000600015
Abstract: purpose: to report a case of erdheim-chester disease with bilateral orbital involvement and to inform ophthalmologists about this uncommon condition. methods: case report. a 41-year-old man presented with proptosis, lid xanthelasmas, disc edema and extraocular muscle restrictions. he was suspected of having a systemic lymphoma and had already been submitted to a retroperitoneal biopsy, however, the diagnosis was still uncertain. results: the patient was evaluated by computed tomography and magnetic resonance imaging of the orbit. orbital pseudotumor was suspected and a biopsy of the orbital lesion initially failed to provide the correct diagnosis which was only made after detailed analysis of the clinical and imaging findings, and review of the histopathologic studies. the systemic diagnosis was made after the orbital diagnosis although the patient had been extensively investigated for more than one year. conclusions: erdheim-chester disease is a rare idiopathic systemic condition characterized by a xanthogranulomatous process involving retroperitoneum, heart, lungs, bone and other tissues. the condition is often fatal due to renal or cardiovascular complications. ocular findings are rare but may be very helpful for the diagnosis. therefore, ophthalmologists should be aware of the clinical manifestations and imaging findings of this rare disease in order to establish an early diagnosis.
Bilateral orbital involvement in Erdheim-Chester disease: case report  [cached]
Monteiro Mário Luiz Ribeiro,Sampaio César Moreira
Arquivos Brasileiros de Oftalmologia , 2002,
Abstract: Purpose: To report a case of Erdheim-Chester disease with bilateral orbital involvement and to inform ophthalmologists about this uncommon condition. Methods: Case report. A 41-year-old man presented with proptosis, lid xanthelasmas, disc edema and extraocular muscle restrictions. He was suspected of having a systemic lymphoma and had already been submitted to a retroperitoneal biopsy, however, the diagnosis was still uncertain. Results: The patient was evaluated by computed tomography and magnetic resonance imaging of the orbit. Orbital pseudotumor was suspected and a biopsy of the orbital lesion initially failed to provide the correct diagnosis which was only made after detailed analysis of the clinical and imaging findings, and review of the histopathologic studies. The systemic diagnosis was made after the orbital diagnosis although the patient had been extensively investigated for more than one year. Conclusions: Erdheim-Chester disease is a rare idiopathic systemic condition characterized by a xanthogranulomatous process involving retroperitoneum, heart, lungs, bone and other tissues. The condition is often fatal due to renal or cardiovascular complications. Ocular findings are rare but may be very helpful for the diagnosis. Therefore, ophthalmologists should be aware of the clinical manifestations and imaging findings of this rare disease in order to establish an early diagnosis.
"Bone scan in Erdheime Chester disease "
"Eftekhari M,Darakhshan S,Vakili A,Fard Esfahani A
Iranian Journal of Nuclear Medicine , 1999,
Abstract: In spite of very high sensitivity, bone scan is quite nonspecific in evaluation of bony lesions. In interpretation of bone scan special attention to patient’s clinical presentation and correlation with other lab and imaging modalities should be paid. In this report a patient with Erdheim Chester disease a rare and unusual form of lipidosis is presented and the pattern of 99mTc-MDP bone scan and other imaging modalities are discussed.
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.
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.
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.
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