Related Experiment Video

Updated: Sep 29, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Erdheim-Chester disease with prominent pericardial involvement: clinical, radiologic, and histologic findings

Anu Gupta1, Benjamin Kelly, James E McGuigan

  • 1Department of Medicine, University of Florida College of Medicine, Gainesville 32610, USA.

The American Journal of the Medical Sciences
|August 21, 2002
PubMed

Insights

Erdheim-Chester disease (ECD), a rare histiocytosis, can affect the heart and skeleton. This case highlights ECD causing cardiac tamponade and jaundice due to skeletal and pericardial involvement.

Area of Science:

  • Oncology
  • Pathology
  • Radiology

Background:

  • Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis.
  • Skeletal and pericardial involvement are uncommon manifestations of ECD.

Observation:

  • A 30-year-old woman presented with jaundice and hepatic congestion.
  • Cardiac tamponade was identified as the cause of her symptoms.
  • Radiologic studies revealed diffuse osteosclerosis of long bones, sparing epiphyses and axial skeleton.

Findings:

  • Histopathology and immunocytochemistry confirmed ECD.
  • Pericardial biopsy showed xanthogranulomatous lesions with foamy macrophages and fibrosis.
  • Immunohistochemistry was positive for CD68 and negative for CD1a, differentiating from Langerhans cell histiocytosis.

Implications:

  • This case underscores the potential for ECD to cause severe cardiac complications like tamponade.
  • Early diagnosis and recognition of diverse ECD manifestations are crucial for patient management.
  • Understanding ECD's skeletal and cardiac impact aids in developing targeted therapeutic strategies.

More Related Videos

An Intact Pericardium Ischemic Rodent Model07:15

An Intact Pericardium Ischemic Rodent Model

Published on: September 2, 2021

Related Concept Videos

Pericarditis II: Clinical Features and Diagnostic Tests01:19

Pericarditis II: Clinical Features and Diagnostic Tests

Pericarditis is distinguished by inflammation of the pericardium, the fibrous sac that encases the heart. It can be acute, lasting less than six weeks, or chronic, persisting for over three months. Understanding its clinical manifestations and diagnostic findings is crucial for timely and effective management.Clinical ManifestationsWhile pericarditis can be asymptomatic, it usually presents with characteristic symptoms such as:Chest Pain: The most characteristic symptom of pericarditis is chest...
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
Myocarditis II: Clinical Features and Diagnostic Tests01:27

Myocarditis II: Clinical Features and Diagnostic Tests

Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...