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Limited Scleroderma (CREST Syndrome)

Definition


Disease: Limited Scleroderma (CREST Syndrome) Limited Scleroderma (CREST Syndrome)
Category: Other Diseases
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Disease Definition:

Scleroderma is a condition that literally means "hardened skin". Limited scleroderma, which is also called CREST syndrome, is one of the subtypes of scleroderma.

Limited scleroderma could affect the digestive tract and cause serious heart and lung disorders, but the skin changes that are associated with it occur only in the lower arms and legs, and in some cases, on the throat and face.

In some cases, CREST syndrome could cause only minor problems but in other cases, the condition could be life altering and even fatal. What exactly causes this condition is still not known. Managing symptoms and preventing serious complications is what treatment will aim at.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

The signs and symptoms of limited scleroderma usually develop gradually, unlike some varieties of scleroderma which occur rapidly. Some of the signs and symptoms of this condition may include:

Raynaud's Phenomenon:
When small blood vessels in the fingers and toes spasm in response to cold or emotional stress, blocking the flow of blood, Raynaud's phenomenon occurs. The skin of most people, before becoming blue, cold and numb, may turn to white. Usually the skin reddens and may throb or tingle when circulation improves. Raynaud's phenomenon is often one of the earliest signs of limited scleroderma, but many people have Raynaud's only and never develop scleroderma.

Tight, Hardened Skin:
Usually, only the skin of lower arms and legs, including fingers and toes, and sometimes the face and throat are affected by limited scleroderma. Because of being pulled taut over underlying bone, the skin may look shiny. Opening the mouth or bending the fingers could become difficult.

Red Spots or Lines on the Skin:
The swelling of tiny blood vessels (telangiectasias) near the skin's surface causes these small red spots or lines. These spots or lines usually occur on the hands and face, and aren't painful.

Bumps Under the Skin:
Tiny calcium deposits (calcinosis) could develop under the skin, mostly on the elbows, knees and fingers, due to limited scleroderma. These deposits could be seen and felt, which could be tender or become infected.

Swallowing Difficulties:
Usually, people who have limited scleroderma experience problems with their esophagus, which is the tube that connects the mouth and stomach. Swallowing could become difficult due to the poor functioning of the muscles in the upper and lower esophagus, and the person may develop conditions such as heartburn, inflammation and scarring of esophageal tissues due to the backing up of stomach acids up into the esophagus.

Causes:

In limited scleroderma, the immune system stimulates the production of too much collagen, which is a key ingredient in connective tissue. This overproduction of collagen will build up in the skin and internal organs, impairing their function. Because of this, limited scleroderma is believed to be an autoimmune disorder.

Sex plays a role in limited scleroderma; women are much more likely to develop limited scleroderma than men are.
Genetic factors may also play a role because the risk of developing limited scleroderma will be increased if one of the family members has an autoimmune disease, such as lupus, Hashimoto's disease or rheumatoid arthritis.
In people with a genetic predisposition to limited scleroderma, toxic substances, such as benzene, trichloroethylene, polyvinyl chloride and silica, could trigger the disease.

Complications

Complications:

The way people feel about their appearance may be affected by the visible signs of limited scleroderma, such as tight, thick skin on the fingers, hands and face. These symptoms could also make everyday tasks such as shaving, or opening a jar, more difficult. Speech could also be affected by this. However, the most serious complications occur beneath the skin.

Ulcers on Fingers and Toes:
Blood flow to the extremities could be obstructed due to severe Raynaud's phenomenon, which could also cause ulcers of the fingers and toes. These ulcers can be difficult to heal. Furthermore, abnormal or narrowed blood vessels combined with severe Raynaud's phenomenon can lead to gangrene of fingers or toes, which may require amputation.

Gastrointestinal Problems:
Chronic heartburn and difficulty swallowing could be caused by the changes in the functioning of esophageal muscles. In case someone’s intestine becomes affected by limited scleroderma, they may experience:

 

  • Weight loss
  • Constipation or diarrhea
  • Malnutrition
  • Bloating after meals


Lung Damage:
A number of problems with the lungs could be caused by limited scleroderma, such as:

Interstitial lung disease: This disorder occurs when excess collagen collects in the tissue between the lungs' air sacs, making the lung tissue stiffer and less able to work properly.

Pulmonary hypertension: The heart will work harder due to increased pressure in the arteries between the heart and lungs, and will eventually become weaker.

Dental Problems:
The mouth could become smaller and narrower due to severe tightening of facial skin, which will put the person at risk of serious dental problems. The risk of tooth decay will be increased because a measurably smaller mouth will make it difficult to brush teeth or have them professionally cleaned. The risk of decay will increase even more because people with limited scleroderma usually don't produce normal amounts of saliva. Also, changes in gum tissue could cause the teeth to become loose or even fall out, and acid reflux could destroy tooth enamel.

Heart Problems:
Abnormal heart rhythms (arrhythmias) could be caused by the scarring of heart tissue, and in rare cases, it could also lead to myocarditis, which is the inflammation of the heart muscle.

Dry Eyes and Mouth:
Limited scleroderma causes many people to have dry eyes and mouth.

Treatments:

There's no cure for limited scleroderma. Treatment focuses on relieving signs and symptoms and preventing complications.

MEDICATIONS:


Blood pressure lowering drugs:
Raynaud's symptoms may be relieved and the increased pressure in the arteries between the heart and lungs reduced by the use of medications that open small blood vessels and increase circulation.

Antacid drugs:
Someone may be suggested medications that reduce the production of stomach acid in case limited scleroderma is causing heartburn.

Drugs that suppress the immune system:
In some people with limited scleroderma, these types of medications have shown to be effective in preventing interstitial lung disease, a condition in which excess collagen collects in the tissue between the air sacs of the lungs.

THERAPY:

Physical therapy:
To help prevent loss of mobility in the finger joints, stretching exercises are quite important. A physical therapist can also show the patient some facial exercises that may help keep the face and mouth flexible as well.

Occupational therapy:
With the help of an occupational therapist, the patient may learn doing things in a different way in case limited scleroderma is making it difficult to perform daily tasks. For instance, to make it easier to care for their teeth, people with this condition could use special toothbrushes and flossing devices.

SURGERY:

Red spots or lines:
The red spots or lines that are caused by swollen blood vessels near the surface of the skin could be reduced by laser surgery.

Calcium deposits:
In some cases, large or painful calcium deposits could be surgically removed.

Gangrene in fingers:
In case skin ulcers progress to gangrene, amputation of fingertips may be necessary.

Prognosis:

Not Available

Expert's opinion

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