Scleroderma

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Edited By Frederick A. Matsen III, M.D., Professor, UW Orthopaedics & Sports Medicine Frederick A. Matsen III, M.D.

Last updated: October 19, 2011

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Basics of scleroderma

Scleroderma is a disease that can affect the skin joints blood vessels and internal organs. The word scleroderma means "hard skin." Most people with scleroderma have problems with their skin and other parts of their bodies.

Prognosis Scleroderma is a chronic disease. This means it may last for months years or for a lifetime. There is no cure for it but it can be treated. With early detection and proper management many people with scleroderma can lead full productive lives.

Incidence

Scleroderma is a rare disease. It affects women two to three times more often than men. The disease usually starts between ages 30 and 50. It is sometimes seen in children and the elderly.

Symptoms

Scleroderma affects everyone differently. It can be mild in some people and more severe in others. Each form of scleroderma affects the body in different ways. Below are some of the ways the disease may affect the body.

Types of scleroderma

There are several different forms of scleroderma (see figure 1). Localized scleroderma affects mainly the skin. It can affect skin in different areas of the body. It may also affect muscles and bone but it does not affect internal organs. This form is usually not as severe as generalized scleroderma. People who develop localized scleroderma usually do not develop generalized scleroderma.

Morphea scleroderma happens when hard oval-shape patches form on the skin. The patches are usually whitish with a purplish ring around them. They usually occur on the trunk but can also occur on the face arms legs and other parts of the body. Morphea often improves by itself over time.

Click to enlarge

Figure 1 - Types of scleroderma

Linear scleroderma is a line of thickened skin that occurs in areas such as the arms legs or forehead. It can occur in more than one area. The line can extend deep into the skin and affect the bones and muscles underneath it. This can affect the motion of joints and muscles as well as the growth of the affected area. When the line forms a long crease on the head or neck it is sometimes called en coup de sabre--the strike of a sword. Linear scleroderma usually occurs in childhood.

Generalized scleroderma affects many parts of the body. It can affect the skin as well as internal body parts such as blood vessels the digestive system (esophagus stomach and bowel) the heart lungs kidneys muscles and joints. The severity of this form depends on the organs affected and how much they are affected. In rare cases scleroderma may affect only some internal system leaving the skin and joints untouched.

There are two types of generalized scleroderma: limited (also called the CREST syndrome) and diffuse.

  • CREST stands for a combination of symptoms: Calcinosis Raynaud's phenomenon Esophageal dysfunction Sclerodactyly and Telangiectasia. This type also known as limited scleroderma usually has a slow onset with the first symptoms appearing 10 to 20 years before the full syndrome occurs. It usually affects the skin on the face fingers and hands. Later on it may affect internal organs such as the esophagus (the tube leading from mouth to the stomach) the lungs and bowels.
  • Diffuse occurs throughout the body. It usually affects the skin as well as other body parts such as the lungs kidneys heart bowels blood vessels and joints. Depending on the areas affected this type can cause problems such as high blood pressure muscle weakness trouble swallowing or shortness of breath. Diffuse scleroderma may progress slowly in some people and more rapidly in others. However with proper management it can usually be controlled.

Skin changes

Skin changes occur in most people with scleroderma. These changes include:

  • hardening and thickening of skin especially on the hands arms and face
  • ulcers on the fingers
  • decrease in hair over the affected area
  • change in skin color

Swelling

Swelling or puffiness of the hands and feet is another common symptom of scleroderma. This often happens in the morning.

You may notice:

  • skin on fingers and toes may look and feel swollen
  • shiny skin
  • usual skin creases disappear
  • tight skin--it may be difficult to make a fist
  • numbness and tingling in fingers

Sclerodactyly

Sclerodactyly means "scleroderma of the digits" (fingers and toes). It usually occurs after the initial swelling goes away.

You may notice:

  • skin on fingers and toes becomes hard and shiny
  • difficulty bending fingers
  • contractures in fingers

Raunaud's phenomenon

Raynaud's phenomenon is a problem of poor blood flow to fingers and toes. Blood flow decreases because blood vessels in these areas become narrow for a short time in response to cold or to emotional stress. Sometimes all it takes to trigger this reaction is walking into a cold room or reaching into the refrigerator. This is an early and common symptom of scleroderma.

You may notice:

  • fingers toes and sometimes the tips of the ears nose or tongue are very sensitive to cold
  • fingers turn bluish or very pale
  • tingling numbness or a cold sensation in the fingers

When the hands warm up the blood vessels open and the skin color returns to normal as the blood supply to the fingers improves.

A note about smoking: If you smoke stop. In addition to all the other health reasons for not smoking smoking can trigger attacks of Raynaud's phenomenon. Contact the American Cancer Society American Heart Association American Lung Association or your doctor to learn about ways to quit smoking.

Telangiectasia

Telangiectasia happens when tiny blood vessels near the surface of the skin show through the skin.

You may notice:

  • small reddish spots on fingers palms face lips and/or tongue

The spots are not harmful and can be hidden with cosmetics.

Calcinosis

Calcinosis happens when small white calcium lumps form under the skin. This is due to scleroderma and is NOT caused by too much calcium in your diet.

You may notice hard white lumps under the skin on fingers or other areas of the body. The lumps may break through the skin and leak a chalky white liquid.

Arthritis and muscle weakness

Arthritis and muscle weakness may also be symptoms of scleroderma. Arthritis happens when joints become painful and swollen. A contracture happens when the skin and tissues around a joint become tight and hard causing the joint to tighten into a bent position. This can happen in the hands and other parts of the body.

You may notice:

  • pain, stiffness, swelling, warmth and tenderness in joints
  • general fatigue
  • weak muscles often in shoulder or hip

Sjogren's syndrome

Sjogren's syndrome is a decrease in secretions from the tear ducts salivary glands and other areas of the body such as the vagina. This happens if scleroderma affects the glands that produce these fluids.

You may notice:

  • dry eyes and mouth
  • trouble speaking or swallowing food
  • dryness in the vagina or other areas of the body
  • increase in dental cavities

Digestive problems

The digestive system includes the mouth esophagus stomach and bowels. Scleroderma can weaken the esophagus and the bowels. It can also cause a build-up of scar tissue in the esophagus which narrows the tube.

You may notice:

  • difficulty swallowing
  • heartburn
  • bloating
  • nausea or vomiting
  • weight loss
  • diarrhea or constipation

Heart and lung problems

Scleroderma may cause the heart to slow down and in some cases can lead to heart failure or other problems. When the lungs are affected they cannot function as well.

You may notice:

  • shortness of breath
  • a persistent cough
  • chest pain

Kidney problems

Scleroderma can cause high blood pressure and kidney failure. If not treated this can be a serious problem. You should be aware of the signs of kidney problems listed here.

You may notice:

  • headache
  • shortness of breath
  • visual disturbances
  • chest pain
  • mental confusion

If you notice any of these signs call your doctor right away so the problem can be treated.

Causes

The cause of scleroderma is unknown. It is not contagious so you can't catch it from someone or give it to anyone. It is not inherited or passed on from one generation to the next except in rare instances.

We do know that in scleroderma the body produces too much of a protein called collagen. This excess collagen is deposited in the skin and in body organs. This causes thickening and hardening of the skin and affects the function of internal organs.

Scientists think the body's immune system plays a part in causing excess collagen deposits. The immune system your body's natural defense against disease normally develops antibodies. Antibodies are protective substances in the blood that fight off invading organisms (like harmful bacteria and viruses). But sometimes the antibodies attack the body's healthy tissue instead. This abnormal process is called an autoimmune reaction.

We know that the small blood vessels are damaged in scleroderma. There may be a connection between the build-up of excess collagen and blood vessel changes. Researchers are trying to find the answer to this as well as to the immune system's role in the disease.

Diagnosis

The first step in diagnosis is a thorough physical examination and history of past and present symptoms.

Diagnostic tests

Laboratory tests and other studies may also be needed to help determine if you have scleroderma and whether it is present in internal organs. These tests can also rule out other diseases but no single test will prove the presence of scleroderma.

Tests may include a biopsy during which a small piece of skin is removed and examined under a microscope. The doctor may also check the esophagus to detect digestive problems. Tests to see whether the immune system is working normally are usually done on a small sample of blood. There are also tests which help detect early lung problems.

Health care team

Since scleroderma affects everyone differently your doctor may need to see you more than once. You may also need to see other doctors such as an arthritis specialist or a skin specialist.

Treatment

After studying your symptoms test results and the overall pattern of your illness your doctor will suggest a treatment program to manage the disease.

This program will include:

  • medication
  • exercises
  • joint protection techniques
  • skin protection techniques
  • stress management

Diet

No diet will cure scleroderma but you should eat balanced meals and stay at a sensible weight. If you have trouble swallowing eat slowly and chew thoroughly. Also drink water or another beverage to soften food. Eat high fiber foods to help cut down on constipation. Try eating six small meals a day rather than three large ones so food digests better.

To help prevent stomach problems:

  • Avoid foods that cause heartburn or gas such as spicy foods.
  • Use antacids for relief from heartburn. Do not lay down for about four hours after a large meal.
  • Eat your largest meal in the middle of the day rather than close to bedtime.
  • Raise the head of your bed by putting six-inch blocks under the end. This will help keep stomach acid from washing into your esophagus while you sleep.

Figure 2 - Finger stretch

Exercise and therapy

Regular exercise helps improve overall health and fitness. For people who have scleroderma it also helps keep the skin and joints flexible maintain better blood flow and prevent contractures. General exercise such as swimming cycling or walking keeps you fit and flexible. Special range of motions exercises help keep skin and specific joints flexible. These should be practiced twice daily.

You can do range-of-motion exercises by yourself or with help from a therapist or family member. Examples of range of motion exercises include:

  • stretching your fingers on a flat table top to help keep them from becoming stiff or fixed in one position (see figure 2)
  • slowly opening your mouth as wide as you can to keep the facial area flexible

A physical or occupational therapist or other health professional can help you with these and other exercises.

Medications

Although there's not yet a cure for scleroderma there are many drugs that help control it. Some work well for some people and not at all for others so what someone else is taking may not be right for you.

  • Aspirin may be used in large doses to treat joint pain and swelling.
  • Nonsteroidal anti-inflammatory drugs (NSAIDs) work like aspirin. They may be used to treat joint pain and swelling.
  • Steroids are synthetic forms of a naturally occurring hormone in the body. They are sometimes used to treat muscle problems. The side effects of these drugs usually outweigh any slight benefit they might produce.
  • Antacids may be used to treat heartburn and to protect the esophagus. You may also be given drugs to decrease stomach acid protect the stomach or improve intestinal motion.
  • Blood pressure medication may be used to treat high blood pressure. It is extremely important to take the medicine the doctor prescribes for you even if you are feeling fine.High blood pressure is known as the "silent" disease and has no symptoms. Taking the medicine faithfully is the only way to keep it under control.
  • Drugs that increase blood flow to your fingers and toes may be used to treat Raynaud's phenomenon.
  • Other drugs may be used to treat the skin or to control the disease.

Caution: Never change your medications without first talking to your doctor.

Strategies for coping

Most of what can be done to manage scleroderma depends on your own strong desire not to let it get the best of you and on your common-sense approach to treatment.

Think of yourself as being in a partnership with your doctor. You both must work to keep the illness under control. Don't be afraid to ask questions. If there's something you don't understand ask the doctor to explain. The better you understand the disease the more actively you can help yourself feel better.

Skin protection

The goal of skin protection is to keep a good supply of blood flowing to your skin and to protect skin from injury. Dressing warmly will help do this. Keeping your body warm helps open the blood vessels in your arms hands legs and feet. Here are some tips for keeping warm:

  • Wear gloves especially if you have Raynaud's phenomenon.
  • Always wear a hat in cold weather since much body heat is lost from an uncovered head.
  • Cover your face and ears with a scarf.
  • During cool weather wear thin cotton garments next to the skin. The cotton layer "moves" the sweat away from the skin and keeps the sweat from cooling your body
  • Wear wool: it's warmer than synthetic cloth like orlon.
  • Wear many thin layers: they'll keep you warmer than one thick garment.
  • Wear loose-fitting boots and shoes that won't cut off the blood supply and will allow you to wear warm thermal socks or layers of socks.

Click to enlarge

Figure 3 - Wrist splint

Other ways to protect your skin:

  • Use a cold water room humidifier to keep skin moist.
  • Avoid using strong detergents or other substances that irritate your skin.
  • Try soap creams and bath oils that are designed to prevent dry skin until you find the ones that give you the best results.
  • Enlist help from family and friends. In the winter for example let someone else get the paper from outside or start the car on a cold day. The same goes for reaching into the freezer or doing tasks that require putting your hands into cold water.

Joint protection

Joint protection means protecting swollen and painful joints from stresses and strains that can make them hurt more. Lifting or carrying heavy objects for example can strain and hurt your joints.

Joint protection includes learning to perform daily activities in ways that will help your joints rather than strain them. Physical and occupational therapists can show you new ways to do activities such as opening doors and drawers getting out of chairs carrying packages ironing clothes and brushing teeth.

Joint protection may also include resting individual joints in removable lightweight splints to help control inflammation (see figure 3). Splints should be well padded to avoid pressure on any areas of the skin.

There are many devices that reduce stress on painful joints which you can purchase or make at home.

Family and friends

Scleroderma should not stop you from having a loving relationship or having sex. Yet because of the illness there may be some emotional and physical concerns to consider.

Scleroderma may change the way you feel about yourself. You may feel you're not as attractive or fun as you used to be. You may feel less sure about your relationship with your partner. Yet while scleroderma may change the way you look to some degree it does not have to change the person you are.

Also consider your partner's feelings. If your partner is concerned about the fatigue discomfort pain and emotional stress the illness causes you he or she may hesitate to have sex. Discuss these feelings with your partner. Through honest sharing you can find ways to solve these problems.

Although sex can play an important part in a relationship it's love and caring that enrich the relationship. If sexual attraction decreases it does not mean that love has decreased also.

There are many things you can do to handle physical problems that may arise. If fatigue is a problem either before or during sex try making love at different times during the day. Also pace yourself while making love to save energy.

If joint pain is a problem try using different positions while making love. A warm bath beforehand and/or a waterbed or electric blanket may also be helpful.

Some men may have problems with impotence due to the illness. Talk to your doctor about ways to treat this problem.

Some birth control methods such as a diaphragm or condom may be physically difficult to use. In such cases your partner may be able to help you insert or apply the device.

Also some birth control pills may cause problems for women with poor blood flow. Talk to your doctor before taking these pills and ask about birth control methods that will work for you.

Stress

There are emotional and social problems that come with having a chronic disease. Even if you can usually manage the illness there may be times when you feel overwhelmed by problems.

Emotional stress plays a part in reducing blood flow. To help reduce the effects of stress you should:

  • Get enough sleep. You may need to take short naps during the day.
  • Try to avoid situations that make you tense.
  • Try to keep feelings of anxiety and fear from getting the best of you.
  • Express your fears and anger about what is happening to you. It is often helpful to confide in family friends your doctor or a counselor. Family members also benefit from such open talks. If they can understand your problems and your feelings they can help you deal with them.
  • Ask your doctor to refer you to a social worker counselor support group or community mental health center.
  • Contact your local chapter of the Arthritis Foundation for information about resources in your community.
  • Look into biofeedback training. It may help control Raynaud's phenomenon. Biofeedback may also help reduce pain and promote relaxation. Talk to your doctor about whether it would be helpful to you and where to get this training.

Climate

A warmer climate will not cure scleroderma but it may cut down on the occurrence of Raynaud's phenomenon and reduce the risk of developing ulcers on your fingers. If you consider moving to a warmer climate you'll need to decide if the benefits outweigh the expenses and complications of relocating.

Resources

For more information contact the Scleroderma Foundation.

Credits

Some of this material may also be available in an Arthritis Foundation brochure. 

Adapted from the pamphlet originally prepared for the Arthritis Foundation by Sergio Jimenez M.D. E. Carwile LeRoy M.D. and Virginia Steen M.D. This material is protected by copyright.