Symptoms of Scleroderma

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Scleroderma, or systemic sclerosis, is a chronic disease that gets worse with time. The most common symptoms of this condition are skin problems, joint and muscle pain, blood vessel narrowing, gastrointestinal problems and calcium deposits in connective tissues. Up to 300,000 Americans have scleroderma according to The Scleroderma Foundation.

What Is Scleroderma?

Scleroderma literally means “hard skin.” It comes from the Greek terms “sclerosis” (hardness) and “derma” (skin). Some people with this condition have localized symptoms, which means symptoms affect only part of the body. Others may have a systemic form of this condition where symptoms will affect the entire body.

Scleroderma causes the body's immune system to malfunction and produce too much collagen, the main substance the body needs for forming connective tissues, which leads to skin and tissue thickening (fibrosis) and scarring.

There is no known cause for scleroderma, but researchers consider it an autoimmune disease where genetics are thought to play a role in its development.

Currently, there are no treatments to help control the overproduction of collagen. Treatment is aimed at relieving symptoms, slowing down the disease’s progression, and preventing complications and disability with medications, diet, lifestyle changes and complementary therapies.

Getting a Diagnosis for Scleroderma

Common Symptoms

Scleroderma affects people differently. The following are the most common symptoms associated with this disorder.

Skin Changes

Scleroderma skin changes include skin hardening of hands, arms, and the face and, sometimes, the torso and legs. Skin thickening can make fingers and toes hard, shiny and difficult to bend. Changes to skin color and hair loss in affected areas and sores on the fingers may also occur. Swelling and puffiness can affect hands and feet, especially in the morning upon waking.

Muscle and Joint Pain

Muscle pain and stiffness are often early signs of scleroderma. Joint pain and stiffness are also common, as is tendon pain. As the disease progresses, muscle loss and weakness may develop. 

Upper and Lower GI Problems

Scleroderma causes problems in the upper GI tract, including fibrosis and acid reflux. Severe reflux diseases may also contribute to lung scarring, bleeding, and narrowing of the esophagus (the muscular tube that carries food from the mouth to the stomach), and an increased risk for esophagus cancer.

As the disease progresses, swallowing and stomach emptying problems are possible. Some people may develop GAVE syndrome, a condition resulting in red streaked areas in the stomach from widened blood vessels. Affecting more than 5 percent of people with systemic scleroderma, GAVE increases the risk of stomach cancer and may to lead to anemia (low red blood cell counts).

In the lower GI tract, scleroderma may affect the intestines by promoting harmful bacteria, slowed movement of food, and reduction of food absorption. Lower GI problems may result in weight loss, cramping, constipation, diarrhea, malnutrition, and fecal incontinence (leakage).

Blood Vessel Narrowing

A condition called Reynaud’s Phenomenon is often associated with scleroderma. Reynaud’s causes poor blood flow to fingers and toes when blood vessels become narrow in response to cold temperatures and stressful situations.  

Some people with scleroderma may develop telangiectasia, which causes tiny blood vessels to widen and show through the skin. Small, red, but harmless spots may appear on the hands and fingers, face, lips, and tongue. 

Basic Facts of Raynaud's Phenomenon

Calcium Deposits Lumps

Calcinosis affects up to a quarter of people with scleroderma, causing small white calcium deposits to form under the skin. These lumps may break through the skin and leak a white substance. Open cuts can become infected.

Other Symptoms

In addition to the most common symptoms, the following symptoms may also affect people with scleroderma:

  • Persistent cough
  • Constipation
  • Shortness of breath
  • Chronic fatigue
  • Weight loss
  • Hair loss
  • Sjogren’s syndrome, which affects up to 20 percent of people with scleroderma and causes dry eyes and mouth

Complications

Scleroderma may cause complications that range from mild to life-threatening due to lack of treatment or when treatments fail.

Complications may include:

  • Restricted movement from skin tightening to joint and muscle problems
  • Damage to fingertips and toes from Reynaud’s, including flesh ulcers, and gangrenes (tissue death) which could lead to amputation
  • Lung complications, including breathing problems due to pulmonary hypertension and lung scarring
  • Kidney damage related to scarring and blood vessel constriction 
  • Heart problems, including abnormal heartbeat and congestive heart failure, from heart tissue scarring and inflammation
  • Dental problems related to dry mouth and acid reflux
  • Sexual dysfunction, including male erectile dysfunction and in women, decreased lubrication and a constricted vaginal opening.
  • Underactive thyroid gland, causing hormonal changes that slow down metabolism
  • Under-active intestines
  • A sluggish esophagus

Death associated with scleroderma is generally linked to heart, kidney, or lung problems.

A Word From Verywell

Treatment for scleroderma has improved over the last two decades due to better and stronger medications. In addition, the search for new drugs to stabilize collagen production is one of the most productive areas of scleroderma research, so there is hope for people with this life-changing condition.

Anyone who has symptoms that affect daily living should talk to their doctor about adjusting their treatment plan. Moreover, it is important to seek out support in order to better cope with scleroderma. The Scleroderma Foundation is a good starting point for finding a support group

Tips for Living Well With Scleroderma
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Article Sources
  • Cleveland Clinic. Scleroderma: An Overview. Updated September 30, 2015.

  • Henderson W. Scleroderma News. 7 Scleroderma Complications. Published August 24, 2017.

  • National Institute of Arthritis and Musculoskeletal and Skin Diseases. Scleroderma. Last Reviewed August 30, 2016.

  • National Institute of Arthritis and Musculoskeletal and Skin Diseases. What is Raynaud’s phenomenon? Updated October 30, 2016.

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