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Case Report

EJMCR. 2019; 3(3): 102-103

Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease

Rita Valerio Alves,Ivan Luz,Rita Calixto,Paulo Santos,Patricia Barreto,Hernani Goncalves,Mario Gois,Helena Viana,Ana Vila Lobos.

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Background: Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytoses with multiorgan xanthomatous infiltration of tissues by histiocytes, surrounded by fibrosis. Case presentation: A 68-year-old Caucasian female was admitted to the emergency department for dyspnea. The patient was given empiric antibiotic therapy with Piperacilin/Tazobactam, but after 10 days the patient underwent respiratory failure accompanied with worsening of the renal function, noninvasive ventilation and diuretics for perfusion were given, but the treatment was unresponsive. Kidney Computed Tomography showed enlargement of both kidneys, suggesting an inflammatory process. A renal biopsy was performed which confirmed the diagnosis of ECD. Conclusion: It is an overlooked diagnosis due to its rarity, variable presentation, and the subsequent manifestations may also develop after several years.

Key words: End-stage renal disease, Erdheim-Chester disease, hemodialysis, histiocytosis, non-Langerhans cell, case report.

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