EJMCR |

Case Report

Volume: 3 | Issue: 3 | Published: Oct 01, 2019 | Pages: 102 - 103 | DOI: 10.24911/ejmcr/173-1550319628

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

Article Info

Authors

Rita Valerio Alves

Nephrology Department, Centro Hospitalar Medio Tejo, Torres Novas, Portugal

ORCID

Ivan Luz

Nephrology Department, Centro Hospitalar Medio Tejo, Torres Novas, Portugal

Rita Calixto

Internal Medicine Department, Unidade Local de Saude do Baixo Alentejo, Beja, Portugal

Paulo Santos

Nephrology Department, Centro Hospitalar Medio Tejo, Torres Novas, Portugal

Patricia Barreto

Nephrology Department, Centro Hospitalar Medio Tejo, Torres Novas, Portugal

Hernani Goncalves

Nephrology Department, Centro Hospitalar Medio Tejo, Torres Novas, Portugal

Mario Gois

Nephrology Department, Centro Hospitalar Universitario Lisboa Central, Lisbon, Portugal

Helena Viana

Nephrology Department, Centro Hospitalar Universitario Lisboa Central, Lisbon, Portugal

Ana Vila Lobos

Nephrology Department, Centro Hospitalar Medio Tejo, Torres Novas, Portugal

Publication History

Received: February 16, 2019

Accepted: September 19, 2019

Published: October 01, 2019

Abstract

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, green

Pubmed Style

Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos. Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. EJMCR. 2019; 01 (October 2019): 102-103. doi:10.24911/ejmcr/173-1550319628

Web Style

Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos. Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. https://ejmcr.com/articles/1472 [Access: December 25, 2024]. doi:10.24911/ejmcr/173-1550319628

AMA (American Medical Association) Style

Vancouver/ICMJE Style

Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos. Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. EJMCR. (2019), [cited December 25, 2024]; 01 (October 2019): 102-103. doi:10.24911/ejmcr/173-1550319628

Harvard Style

Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos (2019) Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. EJMCR, 01 (October 2019): 102-103. doi:10.24911/ejmcr/173-1550319628

Chicago Style

Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos. "Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease." 01 (2019), 102-103. doi:10.24911/ejmcr/173-1550319628

MLA (The Modern Language Association) Style

Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos. "Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease." 01.October 2019 (2019), 102-103. Print. doi:10.24911/ejmcr/173-1550319628

APA (American Psychological Association) Style

Rita Valerio Alves, Ivan Luz, Rita Calixto, Paulo Santos, Patricia Barreto, Hernani Goncalves, Mario Gois, Helena Viana, Ana Vila Lobos (2019) Erdheim-Chester disease: a case report with rare presentation of a catastrophic disease. , 01 (October 2019), 102-103. doi:10.24911/ejmcr/173-1550319628