CREST Syndrome

1 What is Limited Scleroderma (CREST Syndrome)?

Limited scleroderma, also known as CREST syndrome is a type of scleroderma which is diseased condition characterized by skin hardening and induration. In this condition, only some parts of the body like lower arms, legs and sometimes face or throat are affected.

The disease may also affect the digestive tract. Although the problems associated to this disease are not lethal but if it spreads to the lungs and heart, it may be serious.

There is no known treatment for the disease but careful management can help recovering symptoms and preventing complications.

2 Symptoms

Limited scleroderma (CREST Syndrome) usually occurs briskly but the symptoms develop slowly which includes: 

  1. Tight and hardened skin.
  2. Usually affects the lower arms and legs including fingers and toes.
  3. Difficulty in bending fingers. 
  4. Raynaud phenomenon resulting in spasms in fingers and toes due to cold or emotional stress, thus blocking the blood flow. 
  5. Swallowing difficulties due to dysmotility.
  6. Red spots or lines on skin due to telangiectasia.
  7. Poor muscle functioning of the upper and lower esophagus which makes swallowing difficult thus resulting in entry of the stomach acids into the esophagus. 

Limited scleroderma is also known as CREST syndrome, which stands for:

C- Calcinosis for calcium deposition under skin and tissue. This may result in painful infuriation that can cause irritation or break the skin.

R- Raynaud's Phenomenon: Raynaud’s disease is associated with numbness of hands in cold temperature and can be uncomfortable.

E- Esophageal dysmotility: causing heartburn usually seen in CREST patient. 

S- Sclerodactyly: causing thickening of skin.

T- Telangiectasia: resulting in inflamed blood vessels. This condition is usually curable. 

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3 Causes

Limited scleroderma (CREST syndrome) is caused by changes in the body's own immune system.

This disease is considered to be an autoimmune disorder, in which body’s own immune system works against it. The immune system produces collagen (a key component of connective tissue) in excessive amount leading in its deposition in the skin and internal organs thus damaging their functions.

4 Making a Diagnosis

can be difficult to diagnose like any other autoimmune disease. 

One may require consulting various doctors as this disease show variable symptoms, i.e., one should consult a rheumatologist for arthritis and other musculoskeletal disorders, a dermatologist for skin disease and a pulmonologist for lung disorder. The doctor will enquire about the patient’s condition thoroughly, so, it is advised that one must keep in mind the important information like details about symptoms or family medical history. The doctor may ask you the following questions:

  • What symptoms do you have and when did they first appear?
  • Do symptoms change with time?
  • Do you have heartburn condition?
  • If trouble in swallowing? 
  • Whether having extra fatigue or achiness?
  • Is there any change in the color of finger during cold?
  • Weight loss without any exercise? 
  • Any medical history?

There exists a wide variation in signs and symptoms as they may resemble other diseases like polymyositis, lupus, and rheumatoid arthritis. Diagnosis can be done by looking at the signs and symptoms followed by certain tests as described below, to rule out other diseases:

  • Lab tests: Blood test to detect the antibodies present.
  • Skin biopsy: Doctors may also remove the small patch of skin to examine it under the microscope. 

5 Treatment

Limited scleroderma (CREST syndrome) has no cure therefore, the main focus of the treatment lies on preventing complications and reducing the symptoms of the disease.

Medication can also be prescribed in certain cases:

  • Topical antibiotics:  If the affected area becomes infected then one can opt for topical antibiotics and bandages. 
  • Oral or intravenous antibiotics can be used if topical treatment does not work.
  • Antacid drugs could be taken to reduce the stomach acid.
  • Blood pressure lowering drugs to reduce the Raynaud's symptoms. These drugs help in opening up the blood vessels.
  • Immuno-suppressants: These drugs have been proven to prevent the excessive collagen production.

Other therapies:

Stiff, painful joints and hard skin are common in this disease therefore, understanding the proper way of exercising may help in maintaining the flexibility and strength.

  • Physical therapy: The loss of mobility of finger joints can be repaired by performing regular stretching exercise. Facial exercises can help in keeping your face and mouth flexible.
  • Occupational therapy: If one is finding difficulty while doing routine tasks, he/she can opt for occupational therapy. The therapist will help the patient in learning ways to perform work which may involve using special devices such as use of special toothbrushes and flossing device. 
  • Surgery can be performed in some cases for instance in case of calcium deposition, red spots or gangrene in fingers.
  • Laser surgery could also be employed to remove the red spots lines near the surface skin.
  • Amputation may be opted if the skin ulcer leads to gangrene. It involves removal of the affected part.

6 Lifestyle and Coping

Lifestyle modifications are necessary in order to manage the symptoms of Limited scleroderma (CREST syndrome):

  • One can reduce the symptoms of Raynaud by wearing gloves whenever going out in cold weather or while using freezers. 
  • Keeping yourself warm during cold temperature could be helpful. One can wear warmers, socks, caps and well-fitting boots and shoes. 
  • Stop smoking, as the nicotine make the Raynaud symptoms worse and smoking can also result in heartburn.
  • Regular exercise is helpful for keeping your body flexible and powerful. 
  • Changing your eating habits such as: 
  1. Opting for soft, moist food so that one can swallow it easily. 
  2. Eating small meals frequently can help in reducing acid reflux. 
  3. One should avoid eating spicy, or fatty foods, chocolate, caffeine and alcohol. 
  4. Avoid exercise just after eating. 
  5. Sitting upright after meal. 
  • Avoid harsh soap and detergents. Prefer using products that provide you with proper moisture like skin cleansers and bathing gels. 
  • Using rubber gloves while washing dishes or other cleaning purpose may help. 
  • One can also reduce bathing by taking bath once a day or every other day and using warm water rather than hot water. 
  • Using oil rich moisturizer immediately after washing hands or bathing and applying sunscreen in order to prevent skin damage can be very helpful. 
  • Use humidifier to increase moisture. 
  • Maintaining proper oral hygiene can be helpful.

7 Risks And Complications

There are several risks and complications associated with limited scleroderma (CREST syndrome).

Visible signs of Limited scleroderma may include tightening and thickening of the skin of fingers, hands and face but more complicated symptoms are present under your skin.

  • Gastrointestinal problems: Change in the functioning of esophageal muscle thus leading to difficulty in swallowing which further can lead to other problems like constipation, diarrhea, bloating, unintended weight loss and malnutrition. 
  • Raynaud’s phenomenon: Blocking and swelling of blood vessels lead to red spots and line on the surface of the skin. 
  • Lung damage can result due to accumulation of excess collagen in the tissue between the lungs air sacs leading to breathing problems. 
  • Increased blood pressure makes the heart work harder which eventually makes it weak.
  • Heart problems:  Abnormal heart rhythms are the result of scarring of tissues (heart) which may rarely result in inflammation of  heart muscle (myocarditis). 
  • Dental problems: Tightening of facial skin can create problems while opening mouth for brushing teeth. Enamel can be destroyed and changes in the gum can be induced due to acid reflux. 
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