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Related Concept Videos

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...
Cirrhosis I: Introduction01:23

Cirrhosis I: Introduction

Cirrhosis is a chronic, irreversible liver disease characterized by the widespread replacement of healthy liver tissue with fibrotic scar tissue and the formation of regenerative nodules.Etiology of cirrhosisCirrhosis results from sustained liver injury that triggers progressive fibrosis and structural remodeling. The underlying causes are diverse, encompassing common and less frequent clinical conditions. Regardless of the origin, all causes lead to chronic inflammation, hepatocyte loss, and...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
Healing II: Complications01:24

Healing II: Complications

Complications during healing arise when tissue repair is altered by local or systemic factors. These changes involve abnormal collagen deposition, altered biomechanics, and reduced vascular supply, impairing restoration of normal structure and function.Loss of FunctionScar tissue differs significantly from the original tissue it replaces. In the skin, fibrosis lacks adnexal structures such as hair follicles, sebaceous glands, and sweat glands. Their absence reduces tactile sensitivity, impairs...
Reticular Dermis01:15

Reticular Dermis

The papillary and reticular dermis are the two layers of the dermis. They are made of connective tissue with fibers of collagen extending from one to the other, making the border between the two somewhat indistinct. The dermal papillae extending into the epidermis belong to the papillary layer, whereas the dense collagen fiber bundles below belong to the reticular layer.
Reticular Layer
Underlying the papillary layer is the much thicker reticular layer, composed of dense, irregular connective...
Papillary Dermis01:11

Papillary Dermis

Dermis
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
The papillary layer is made of loose, areolar connective tissue, which means the collagen and...

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Published on: June 16, 2020

[Scleroderma].

O Distler1, S Gay

  • 1Rheumaklinik und Institut für physikalische Medizin, UniversitätsSpital Zürich, Gloriastrasse 25, 8091 Zürich, Schweiz. oliver.distler@usz.ch

Der Internist
|December 25, 2009
PubMed
Summary
This summary is machine-generated.

Scleroderma, or systemic sclerosis, is a complex disease causing fibrosis and vascular issues affecting skin and internal organs. Early diagnosis and understanding its varied manifestations are crucial for managing this challenging condition.

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Area of Science:

  • Rheumatology and Immunology
  • Internal Medicine
  • Dermatology

Context:

  • Scleroderma, also known as systemic sclerosis, is a multisystem autoimmune disease.
  • It is characterized by excessive extracellular matrix deposition (fibrosis) and vascular abnormalities (vasculopathy).
  • Distinguishing systemic sclerosis from localized scleroderma forms like morphea is critical due to differing prognoses.

Purpose:

  • To provide a comprehensive overview of the current understanding of scleroderma.
  • To summarize key aspects including classification, epidemiology, pathogenesis, and clinical manifestations.
  • To review current and emerging therapeutic strategies for systemic sclerosis.

Summary:

  • Systemic sclerosis involves widespread organ damage, including lung, gastrointestinal, kidney, and heart involvement.
  • Key clinical features include organ fibrosis, fingertip ulcers, pulmonary arterial hypertension, and acute renal crisis.
  • The disease presents significant diagnostic and therapeutic challenges due to its heterogeneity and high morbidity/mortality.

Impact:

  • This review aids clinicians in diagnosing and managing scleroderma more effectively.
  • It highlights the need for further research into the pathogenesis and treatment of systemic sclerosis.
  • Improved understanding can lead to better patient outcomes and quality of life for those affected by this rare disease.