Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease
Authors: Rita Valerio Alves , Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos
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.
Keywords: End-stage renal disease, Erdheim-Chester disease, hemodialysis, histiocytosis, non-Langerhans cell, case report.
Authors
Correspondence to:
Nephrology Department, Centro Hospitalar Medio Tejo, Torres Novas, Portugal anarita1990@gmail.com
Publication history:
Received 16 Feb 2019
Accepted 19 Sep 2019
Published online 01 Oct 2019
Published in print 15 Dec 2019
RVA, Luz I, Calixto R, Santos P, Barreto P, Goncalves H, Gois M, Viana H, Lobos AV, . Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. EJMCR. 2019; 3(3): 102-103. doi:
10.24911/ejmcr/173-1550319628
RVA, Luz I, Calixto R, Santos P, Barreto P, Goncalves H, Gois M, Viana H, Lobos AV, . Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. https://www.ejmcr.com/?mno=31835 [Access: April 17, 2024]. doi:
10.24911/ejmcr/173-1550319628
RVA, Luz I, Calixto R, Santos P, Barreto P, Goncalves H, Gois M, Viana H, Lobos AV, . Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. EJMCR. 2019; 3(3): 102-103. doi:
10.24911/ejmcr/173-1550319628
RVA, Luz I, Calixto R, Santos P, Barreto P, Goncalves H, Gois M, Viana H, Lobos AV, . Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. EJMCR. (2019), [cited April 17, 2024]; 3(3): 102-103. doi:
10.24911/ejmcr/173-1550319628
, R. V. A., Luz, I., Calixto, R., Santos, P., Barreto, P., Goncalves, H., Gois, M., Viana, H., Lobos, A. V. & (2019) Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. EJMCR, 3 (3), 102-103. doi:
10.24911/ejmcr/173-1550319628
, Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos, and . 2019. Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. European Journal of Medical Case Reports, 3 (3), 102-103. doi:
10.24911/ejmcr/173-1550319628
, Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos, and . "Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease." European Journal of Medical Case Reports 3 (2019), 102-103. doi:
10.24911/ejmcr/173-1550319628
, Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos, and . "Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease." European Journal of Medical Case Reports 3.3 (2019), 102-103. Print. doi:
10.24911/ejmcr/173-1550319628
, R. V. A., Luz, I., Calixto, R., Santos, P., Barreto, P., Goncalves, H., Gois, M., Viana, H., Lobos, A. V. & (2019) Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. European Journal of Medical Case Reports, 3 (3), 102-103. doi:
10.24911/ejmcr/173-1550319628