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
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
