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Scleroderma

Scleroderma: Introduction

Scleroderma is an ongoing, progressive, autoimmune disease that attacks the connective tissues of the body. Scleroderma means hardening of the skin, and is a potentially serious disease in some cases.

There are two types of scleroderma. Localized scleroderma is the milder form of the disease and affects the skin and musculoskeletal system. Systemic scleroderma is generally more serious and can affect connective tissues in many parts of the body, including the lungs, heart digestive tract, blood vessels, and kidneys, as well as the skin.

The exact cause of scleroderma is not known, but it is classified as an autoimmune disease. In an autoimmune disease, the body's immune system mistakes healthy tissues as foreign and potentially dangerous invaders into the body and attacks them. This process results in an excessive production of a protein called collagen and a hardening of tissues, which can seriously affect many body systems, including the skin, blood vessels and organs.

Scleroderma is a rare disease. Scleroderma is more common in African-Americans than in Whites. Scleroderma is also more common in women than in men.

The symptoms of scleroderma can be mild, moderate, or severe. Typical symptoms affect the fingers and toes and include paleness, coldness, pain, swelling and numbness. A thickening of the skin of the hands and feet may also occur. Serious symptoms and complications can happen with scleroderma, especially systemic scleroderma, also known as CREST syndrome. Complications may include pulmonary fibrosis, kidney damage, hypertension and abnormal heart rhythms. For additional symptoms and more information on complications, refer to symptoms of scleroderma.

Making a diagnosis of scleroderma begins with taking a thorough medical history, including symptoms, and completing a physical examination with a focus on the skin. Diagnostic testing includes a blood test that measures the antibody that the body produces in scleroderma.

A skin biopsy may also be done. In a biopsy, a small piece of affected tissue is examined under a microscope in the laboratory for excessive amounts of collagen. Other tests are performed to evaluate general health and help to determine the extent of scleroderma and if complications, such as kidney damage and pulmonary fibrosis, are present. These may include chest X-ray, urinanalysis, pulmonary function tests, ESR test, and echocardiogram of the heart.

It is possible that a diagnosis of scleroderma can be missed or delayed because some symptoms can be similar to symptoms of other conditions, such as rheumatoid arthritis. For more information on disease and conditions that can mimic scleroderma, refer to misdiagnosis of scleroderma.

Treatment for scleroderma varies depending on the severity of symptoms, the presence of complications, a person's age and medical history, and other factors. Scleroderma cannot be cured, but supportive care can help to reduce symptoms. Treatment can include a combination of medication, lifestyle changes, physical therapy and exercise. For more information on treatment, refer to treatment of scleroderma. ...more »

Scleroderma: Derived from the Greek words "sklerosis," meaning hardness, and "derma," meaning skin, scleroderma literally means hard skin. Though it is ... more about Scleroderma.

Scleroderma: A rare, progressive connective tissue disorder involving thickening and hardening of the skin and connective tissue. There are a number of forms of scleroderma with some forms being systemic (involving internal organs). More detailed information about the symptoms, causes, and treatments of Scleroderma is available below.

Scleroderma: Symptoms

The types and severity of symptoms of scleroderma vary greatly between individuals and the type of scleroderma. Symptom severity can range from mild to severe with disabling and life-threatening complications. Symptoms generally begin in adulthood between the ages of 20 and 40.

Symptoms of the milder form of scleroderma, localized scleroderma, which affects the ...more symptoms »

Scleroderma: Treatments

There is no cure for scleroderma, and the disease generally gets worse over time. People with the more severe form of scleroderma, systemic scleroderma, have a greater risk of developing serious, even-life threatening and fatal complications, such as pulmonary fibrosis, abnormal heart rhythms, kidney failure, hypertension, seizures and heart failure.

However, ...more treatments »

Scleroderma: Misdiagnosis

A diagnosis of scleroderma may be delayed or missed because some symptoms, such as difficulty swallowing and heartburn are vague and similar to symptoms of other diseases and disorders. These include a wide variety of gastrointestinal disorders, such as GERD, heartburn, and gastroenteritis. Symptoms, such as finger swelling, joint stiffness and limited mobility can be similar to ...more misdiagnosis »

Symptoms of Scleroderma

Treatments for Scleroderma

Wrongly Diagnosed with Scleroderma?

Scleroderma: Related Patient Stories

Scleroderma: Deaths

Read more about Deaths and Scleroderma.

Types of Scleroderma

Curable Types of Scleroderma

Possibly curable types of Scleroderma include:

Rare Types of Scleroderma:

Rare types of Scleroderma include:

  • Pericardial effusion associated with scleroderma
  • Erectile dysfunction associated with scleroderma
  • Hypothyroidism associated with scleroderma
  • Primary biliary cirrhosis related to scleroderma
  • Trigeminal neuralgia associated to scleroderma
  • Carpal tunnel syndrome associated to scleroderma
  • more types...»

Diagnostic Tests for Scleroderma

Scleroderma: Complications

Review possible medical complications related to Scleroderma:

Causes of Scleroderma

Read more about causes of Scleroderma.

More information about causes of Scleroderma:

Disease Topics Related To Scleroderma

Research the causes of these diseases that are similar to, or related to, Scleroderma:

Misdiagnosis and Scleroderma

Psoriasis often undiagnosed cause of skin symptoms in children: Children who suffer from the skin disorder called psoriasis can often go...read more »

Scleroderma: Research Doctors & Specialists

Research related physicians and medical specialists:

Other doctor, physician and specialist research services:

Scleroderma: Animations

Prognosis for Scleroderma

Prognosis for Scleroderma: For some people, scleroderma (particularly the localized forms) is fairly mild and resolves with time. But for others, living with the disease and its effects day to day has a significant impact on their quality of life. (Source: excerpt from Handout on Health Scleroderma: NIAMS) ... No cure exists, but timely intervention can improve the quality of life. (Source: excerpt from Connective Tissue Diseases: NWHIC)

Research about Scleroderma

Visit our research pages for current research about Scleroderma treatments.

Statistics for Scleroderma

Scleroderma: Broader Related Topics

Scleroderma Message Boards

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User Interactive Forums

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Article Excerpts about Scleroderma

Handout on Health Scleroderma: NIAMS (Excerpt)

Derived from the Greek words "sklerosis," meaning hardness, and "derma," meaning skin, scleroderma literally means hard skin. Though it is often referred to as if it were a single disease, scleroderma is really a symptom of a group of diseases that involve the abnormal growth of connective tissue, which supports the skin and internal organs. It is sometimes used, therefore, as an umbrella term for these disorders. In some forms of scleroderma, hard, tight skin is the extent of this abnormal process. In other forms, however, the problem goes much deeper, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.

Scleroderma is called both a rheumatic (roo-MA-tik) disease and a connective tissue disease. The term rheumatic disease refers to a group of conditions characterized by inflammation and/or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects the major substances in the skin, tendons, and bones. (Source: excerpt from Handout on Health Scleroderma: NIAMS)

Connective Tissue Diseases: NWHIC (Excerpt)

Scleroderma is an activation of immune cells which produces scar tissue in the skin, internal organs, and small blood vessels. It affects women three times more often than men overall, but increases to a rate 15 times greater for women during childbearing years, and appears to be more common among black women. (Source: excerpt from Connective Tissue Diseases: NWHIC)

Definitions of Scleroderma:

Systemic disorder of the connective tissue; manifested by hardening and thickening of the skin, by abnormalities involving the microvasculature and larger vessels, and by fibrotic degenerative changes in various body organs including the heart, lungs, kidneys, and gastrointestinal tract. - (Source - Diseases Database)

An autoimmune disease that affects the blood vessels and connective tissue; fibrous connective tissue is deposited in the skin - (Source - WordNet 2.1)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Scleroderma as a "rare disease".
Source - Orphanet

 

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