Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

964
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...
964
Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

207
Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum...
207
Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

861
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...
861
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

1.2K
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...
1.2K
Hypersensitivity Reactions: Delayed Hypersensitivity Reactions01:29

Hypersensitivity Reactions: Delayed Hypersensitivity Reactions

268
Delayed-Type Hypersensitivity (DTH), or Type IV hypersensitivity, is a cell-mediated immune response. It occurs when T cells, rather than antibodies, mediate a reaction to specific antigens. It is characterized by a delayed onset (1-2 days) and involves the recruitment of macrophages to the inflammation site.The initiation of a DTH response begins with the sensitization of T cells. During this phase, which lasts at least 1-2 weeks, antigen-specific T cells are activated, clonally expanded, and...
268
Skin Diseases and Disorders01:23

Skin Diseases and Disorders

6.3K
Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
6.3K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Combined large and small airway obstruction is associated with increased exacerbation frequency and poorer symptom control in persistent asthma.

The journal of allergy and clinical immunology. In practice·2026
Same author

A Delphi survey of patients and clinicians on a unified and stratified approach to managing GCA-PMR spectrum disease.

Rheumatology (Oxford, England)·2026
Same author

Editorial: Insights into the diagnosis, pathogenesis and management of Takayasu arteritis.

Frontiers in immunology·2026
Same author

Small Airway Dysfunction May Mediate the Association Between Body Mass Index and Severe Asthma Exacerbations.

The European respiratory journal·2026
Same author

Dual Remission in Patients with Moderate-to-Severe Asthma and CRSwNP Treated with Dupilumab: A 24-Month Real-World Study.

Journal of clinical medicine·2026
Same author

Recurrent fever and association with severe organ involvement, mortality and treatment outcomes in VEXAS syndrome: data from the AIDA Network.

Frontiers in immunology·2026
Same journal

Early brain biopsy in neurological diseases of unknown etiology: Moving the diagnostic clock forward, not skipping the work-up.

European journal of internal medicine·2026
Same journal

Positive urine cultures before neutropenia: Interpreting the organism through the host.

European journal of internal medicine·2026
Same journal

Why rare uninformative findings appear highly specific.

European journal of internal medicine·2026
Same journal

Artificial intelligence in diagnostics and medical decision making.

European journal of internal medicine·2026
Same journal

Diagnosis, treatment and outcomes of euvolemic hyponatremia among geriatric hospitalised patients.

European journal of internal medicine·2026
Same journal

Sex-based differences and clinical outcomes in older adults with Candida bloodstream infection: a post-hoc secondary analysis of a multicentre study.

European journal of internal medicine·2026
See all related articles

Related Experiment Video

Updated: Apr 15, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
10:27

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

25.1K

Erdheim-Chester disease.

Corrado Campochiaro1, Alessandro Tomelleri1, Giulio Cavalli1

  • 1Unit of Medicine and Clinical Immunology, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.

European Journal of Internal Medicine
|April 14, 2015
PubMed
Summary
This summary is machine-generated.

Erdheim-Chester disease (ECD) is a rare histiocytosis often involving multiple organs and linked to BRAF mutations. Current treatments include interferon-alfa, with newer BRAF inhibitors showing promise but requiring further study.

Keywords:
BRAF geneDiagnosisErdheim-Chester diseaseHistiocytosisTreatmentVemurafenib

More Related Videos

Granulocyte-dependent Autoantibody-induced Skin Blistering
12:23

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

11.0K
Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity
11:12

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity

Published on: April 11, 2019

7.6K

Related Experiment Videos

Last Updated: Apr 15, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
10:27

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

25.1K
Granulocyte-dependent Autoantibody-induced Skin Blistering
12:23

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

11.0K
Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity
11:12

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity

Published on: April 11, 2019

7.6K

Area of Science:

  • Rare diseases
  • Histiocytosis
  • Oncology

Background:

  • Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with multiorgan involvement.
  • Patients are typically diagnosed in their fifth decade, with a slight male predominance.
  • Mutations in the BRAF gene and MAPK pathway are implicated in ECD pathogenesis, suggesting a clonal origin.

Purpose of the Study:

  • To review the clinical manifestations, diagnosis, and treatment of Erdheim-Chester disease.
  • To highlight the role of BRAF mutations in ECD.
  • To discuss current and emerging therapeutic strategies.

Main Methods:

  • Literature review of ECD studies.
  • Analysis of clinical features and diagnostic criteria.
  • Evaluation of treatment outcomes for interferon-alfa, anakinra, infliximab, and vemurafenib.

Main Results:

  • ECD presents with diverse symptoms, commonly affecting bones, endocrine system (diabetes insipidus), cardiovascular system (aortic thickening), and retroperitoneum.
  • Cardiovascular and central nervous system involvement are linked to poorer prognosis.
  • Diagnosis requires biopsy confirming CD68+/CD1a-/S100- foamy histiocytes.

Conclusions:

  • Interferon-alfa remains first-line treatment, improving survival.
  • Anakinra and infliximab are alternatives for treatment failure.
  • BRAF inhibitors like vemurafenib show efficacy but require more data on long-term safety and effectiveness.