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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune system...
Inflammatory Bowel Disease III: Crohn's Disease01:25

Inflammatory Bowel Disease III: Crohn's Disease

Crohn’s disease is a chronic, relapsing form of inflammatory bowel disease characterized by segmental, transmural inflammation that can affect any part of the gastrointestinal tract. Its pathogenesis arises from a combination of genetic susceptibility, environmental exposures, epithelial barrier dysfunction, and immune dysregulation. Together, these factors lead to an exaggerated immune response against components of the gut microbiome.Genetic and Environmental InfluencesMultiple genetic...
Skin Diseases and Disorders01:23

Skin Diseases and Disorders

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...
Inflammatory Bowel Disease II: Crohn's Disease01:30

Inflammatory Bowel Disease II: Crohn's Disease

Introduction
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by transmural...
Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Inflammatory Bowel Disease I: Introduction01:26

Inflammatory Bowel Disease I: Introduction

Inflammatory bowel disease is a group of chronic disorders marked by recurrent inflammation of the gastrointestinal tract due to an abnormal immune response against gut microflora. This leads to tissue damage. The two main forms are Crohn’s disease and ulcerative colitis.Crohn’s DiseaseCrohn’s disease is a relapsing inflammatory disorder that can affect any part of the GI tract, from the mouth to the anus. It involves all layers of the bowel wall (transmural) and shows “skip lesions” in which...

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Related Experiment Videos

Autoinflammatory syndromes with a dermatological perspective.

Nobuo Kanazawa1, Fukumi Furukawa

  • 1Department of Dermatology, Wakayama Medical University, Wakayama, Japan. nkanazaw@wakayama-med.ac.jp

The Journal of Dermatology
|August 31, 2007
PubMed
Summary

Autoinflammatory syndromes are distinct systemic inflammatory diseases, often linked to genetic mutations affecting innate immunity. This review summarizes key syndromes and discusses skin disorder pathophysiology due to immune dysregulation.

Related Experiment Videos

Area of Science:

  • Immunology
  • Genetics
  • Dermatology

Background:

  • Autoinflammatory syndromes are systemic inflammatory diseases distinct from infectious, autoimmune, or allergic conditions.
  • Initially defined by hereditary periodic fever syndromes, the concept now includes a broader range of rare and common inflammatory diseases.
  • These conditions share common clinical features involving neutrophil and/or monocyte/macrophage activation.

Purpose of the Study:

  • To review major autoinflammatory syndromes.
  • To discuss the pathophysiology of associated skin disorders.
  • To highlight the role of dysregulated innate immune signaling.

Main Methods:

  • Literature review of autoinflammatory syndromes.
  • Summary of genetic associations.
  • Discussion of clinical features and pathophysiology.

Main Results:

  • Autoinflammatory syndromes encompass hereditary periodic fevers (e.g., Familial Mediterranean Fever) and cryopyrin-associated periodic syndromes (CAPS).
  • The spectrum extends to other conditions like Behçet's disease, Crohn's disease, and psoriatic arthritis.
  • Mutations in genes regulating innate immunity are central to these diseases.

Conclusions:

  • Autoinflammatory syndromes represent a diverse group of disorders rooted in innate immune system dysregulation.
  • Understanding the genetic basis and immune pathways is crucial for diagnosing and managing these conditions.
  • Further research into innate immune signaling is needed to elucidate the pathophysiology of associated skin manifestations.