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SclerodermaScleroderma: Causes, Symptoms, Risk Factors, Types, Complications, Medications and Treatments

Commonly asked questions about Scleroderma:

What is Scleroderma?

It is a set of rare diseases that involve the hardening and tightening of skin and other connective tissues. It only affects the skin in some patients, however, in many others it also affects blood vessels, internal organs and the digestive tract.

What Causes Scleroderma?

According to the Mayo Clinic, it is the result from your body’s overproduction and storage of collagen in body tissues. Collagen is the protein that makes up your body’s skin and other connective tissues. Doctors are unsure of what actually triggers the abnormal collagen production. They only know that the body’s immune system is a factor.

What Risk Factors are Associated with Scleroderma?

Medical News Today states that doctors are unsure of what exactly causes the disease. However, they do know that it is about four times more likely to occur in women than in men. If a person has a family member that has another form of autoimmune disease they may be at an increased risk of developing it.

What are the Different Types of Scleroderma?

The Scleroderma Foundation states that it falls into two major classifications known as localized and systemic sclerosis.

  • Localized: The mildest form, it does not affect the internal organs. Localized scleroderma can be broken down into two smaller sub-categories known as Morphea and Linear.
    • Morphea: Oval-shaped patches of lighter or darker skin, which may be itchy, hairless and shiny. The shapes have a purple border and are white in the middle.
    • Linear: People that are diagnosed with linear may have bands or streaks of hardened skin on the head, face and limbs. It is also able to affect bones and muscles.
  • Systemic Sclerosis: Affects the circulation of blood and the internal organs, especially the kidneys, esophagus, heart and lungs. There are also two smaller sub-categories of systemic scleroderma known as Limited and Diffuse.
    • Limited: Is the least severe type of systemic. It affects the skin on the hands, feet, face, and lower arms and legs. There may be problems with the lungs and the digestive system.
    • Diffuse: Skin thickening occurs more rapidly than in limited as well as involving more skin areas. People affected by diffuse also have a higher risk of the hardening of internal organs.

What are the Signs/Symptoms/Complications of Scleroderma?

The Mayo Clinic provides a list of the most common signs and symptoms, these include:

Signs/SymptomsDescription
SkinMost people experience a hardening and tightening of skin patches. Patches may be shaped like ovals or straight lines, or cover wide areas. The type determines the number, location and size of the patches.
Fingers or ToesOne of the first signs of the disease is an intolerance of cold temperatures or exaggerated emotional distress, which can cause numbness, pain or color changes in the fingers or toes.
Digestive SystemIf your intestinal muscles are having a hard time moving food properly through the intestines, you may have problems absorbing key nutrients.
Heart, Lungs or KidneysThe disease has the ability to affect the function of the heart, lungs or kidneys to varying degrees. If left untreated, these issues may become life-threatening.

The Mayo Clinic also lists a number of severe complications that may occur alongside normal symptoms, such as:

ComplicationsDescription 
FingertipsThe hardening and tightening of skin patches that occurs can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores. In some cases, gangrene and amputation may follow.
LungsScarring of lung tissue can result in a decrease of the lungs’ ability to function as well as difficulty breathing and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs.
KidneysIf it affects your kidneys, you may develop elevated blood pressure as well as an increased level of protein in your urine. The more-serious effect of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
HeartScarring of heart tissue increases your risk of developing an arrhythmia or congestive heart failure, and can cause inflammation of the sac surrounding your heart. It can also cause your heart to wear out by increasing the pressure on the right side.
TeethSevere tightening of facial skin can cause your mouth to become smaller and narrower, which may make it difficult to brush your teeth or to even have a dentist clean them. People who have the disease often don’t produce normal amounts of saliva, so the risk of dental decay increases even more.
Digestive SystemDigestive problems linked with the disease may lead to acid reflux and difficulty swallowing.
Sexual FunctionMen who have the disease often experience erectile dysfunction. It may also affect women, by decreasing sexual lubrication and constricting the vaginal opening.

Is Scleroderma Curable?

Currently there is no cure available. Even though there is not a cure the Scleroderma Foundation says that there are a number of available treatments to assist people with the varying symptoms that they may have.

What Drugs/Medicines are used to Treat Scleroderma?

Below you will find a list of drugs that are used to help treat the disease. Due to there being such a wide variety of symptoms and severity of this condition there is no one specific medicine that is used to treat it. The Scleroderma Research Foundation lists the possible drug groups. They include:

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View Sources

  1. Mayo Clinic – Scleroderma Causes
  2. Medical News Today – Causes and Risk Factors
  3. Scleroderma Foundation – What is Scleroderma
  4. Mayo Clinic – Symptoms and Causes
  5. Scleroderma Foundation – Cure
  6. Scleroderma Research Foundation – Current Treatments

Resources

Carvalho D, Savage C, Black C, Pearson J, IgG antiendothelial cell autoantibodies from scleroderma patients induce leukocyte adhesion to human vascular endothelial cells in vitro. Induction of adhesion molecule expression and involvement of endothelium-derived cytokines, 1996, The Journal of Clinical Investigation, vol.97(1), 111-119

Stratton R, Shiwen X, Martini G, Holmes A, Leask A, Haberberger T, Martin G, Black C, Abraham D, Iloprost suppresses connective tissue growth factor production in fibroblasts and in the skin of scleroderma patients, 2001, The Journal of Clinical Investigation, vol.108(2), 241-250

Pope J, Ouimet J,  Krizova A, Scleroderma treatment differs between experts and general rheumatologists, 2006, TOC, vol.55(1), 138-145