Scleroderma (skleer-oh-DUR-muh) is a group of rare diseases that involve the hardening and tightening of the skin and connective tissues — the fibers that provide the framework and support for your body.
In some people, scleroderma affects only the skin. But in many people, scleroderma also harms structures beyond the skin — such as blood vessels, internal organs and the digestive tract. Signs and symptoms vary, depending on which structures are affected.
Scleroderma affects women more often than men and most commonly occurs between the ages of 30 and 50. While there is no cure for scleroderma, a variety of treatments can ease symptoms and improve quality of life.
Scleroderma's signs and symptoms vary, depending on which parts of your body are involved:
- Skin. Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it's so tight, and movement of the affected area may be restricted.
- Fingers or toes. One of the earliest signs of scleroderma is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, pain or color changes in the fingers or toes. Called Raynaud's phenomenon, this condition also occurs in people who don't have scleroderma.
- Digestive system. In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients if their intestinal muscles aren't moving food properly through the intestines.
- Heart, lungs or kidneys. Rarely, scleroderma can affect the function of the heart, lungs or kidneys. These problems can become life-threatening.
Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin.
Although doctors aren't sure what prompts this abnormal collagen production, the body's immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen.
Scleroderma occurs much more often in women than it does in men. Choctaw Native Americans and African-Americans are more likely than Americans of European descent to develop the type of scleroderma that affects internal organs.
Scleroderma complications range from mild to severe and can affect your:
- Fingertips. The variety of Raynaud's phenomenon that occurs with scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores (ulcers). In some cases, gangrene and amputation may follow.
- Lungs. Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).
- Kidneys. When scleroderma affects your kidneys, you can develop an elevated blood pressure and an increased level of protein in your urine. More-serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
- Heart. Scarring of heart tissue increases your risk of abnormal heartbeats (arrhythmias) and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart (pericarditis). Scleroderma also can raise the pressure on the right side of your heart and cause it to wear out.
- Teeth. Severe tightening of facial skin can cause your mouth to become smaller and narrower, which may make it hard to brush your teeth or to even have them professionally cleaned. People who have scleroderma often don't produce normal amounts of saliva, so the risk of dental decay increases even more.
- Digestive system. Digestive problems associated with scleroderma can lead to acid reflux and difficulty swallowing — some describe feeling as if food gets stuck midway down the esophagus — as well as bouts of constipation alternating with episodes of diarrhea.
- Sexual function. Men who have scleroderma often experience erectile dysfunction. Scleroderma may also affect the sexual function of women, by decreasing sexual lubrication and constricting the vaginal opening.
You'll probably first bring your symptoms to the attention of your family doctor, who may refer you to a rheumatologist — a doctor specializing in the treatment of arthritis and other diseases of the joints, muscles and bone. Because scleroderma can affect many organ systems, you may need to see a variety of medical specialists.
What you can do
Appointments can be brief. To make the best use of the limited time, plan ahead and write lists of important information, including:
- Detailed descriptions of all your symptoms
- A list of all your medications and dosages, including nonprescription drugs and supplements
- Questions for the doctor, such as what tests or treatments he or she may recommend
What to expect from your doctor
Your doctor may ask some of the following questions:
- Do your fingers become numb or change colors when you get cold or upset?
- Do you regularly experience heartburn or swallowing problems?
- Have your parents or siblings ever had similar signs and symptoms?
Because scleroderma can take so many forms and affect so many different areas of the body, it can be difficult to diagnose.
After a thorough physical exam, your doctor may suggest blood tests to check for elevated blood levels of certain antibodies produced by the immune system. He or she may remove a small tissue sample (biopsy) of your affected skin so that it can be examined in the laboratory for abnormalities.
You may also need breathing tests (pulmonary function tests), a CT scan of your lungs and an echocardiogram of your heart.
In some cases, the skin problems associated with scleroderma fade away on their own in three to five years. The type of scleroderma that affects internal organs usually worsens with time.
No drug has been developed that can stop the underlying process of scleroderma — the overproduction of collagen. But a variety of medications can help control scleroderma symptoms or help prevent complications. To accomplish this, these drugs may:
- Dilate blood vessels. Blood pressure medications that dilate blood vessels may help prevent lung and kidney problems and may help treat Raynaud's disease.
- Suppress the immune system. Drugs that suppress the immune system, such as those taken after organ transplants, may help reduce scleroderma symptoms.
- Reduce stomach acid. Medications such as omeprazole (Prilosec) can relieve symptoms of acid reflux.
- Prevent infections. Antibiotic ointment may help prevent infection of fingertip ulcers caused by Raynaud's phenomenon. Regular influenza and pneumonia vaccinations can help protect lungs that have been damaged by scleroderma.
- Relieve pain. If over-the-counter pain relievers don't help enough, you can ask your doctor to prescribe stronger medications.
Physical or occupational therapists can help you to:
- Manage pain
- Improve your strength and mobility
- Maintain independence with daily tasks
Used as a last resort, surgical options for scleroderma complications may include:
- Amputation. If finger ulcers caused by severe Raynaud's phenomenon have developed gangrene, amputation may be necessary.
- Lung transplants. People who have developed high blood pressure in the arteries to their lungs (pulmonary hypertension) may be candidates for lung transplants.
You can take a number of steps to help manage your symptoms of scleroderma:
- Stay active. Exercise keeps your body flexible, improves circulation and relieves stiffness. Range-of-motion exercises can help keep your skin and joints flexible.
- Don't smoke. Nicotine causes blood vessels to contract, making Raynaud's phenomenon worse. Smoking can also cause permanent narrowing of your blood vessels. Quitting smoking is difficult — ask your doctor for help.
- Manage heartburn. Avoid foods that give you heartburn or gas. Also avoid late-night meals. Elevate the head of your bed to keep stomach acid from backing up into your esophagus (reflux) as you sleep. Antacids may help relieve symptoms.
- Protect yourself from the cold. Wear warm mittens for protection anytime your hands are exposed to cold — even when you reach into a freezer. When you're outside in the cold, cover your face and head and wear layers of warm clothing.
Meditation and relaxation techniques can help you cope with the frustrations of scleroderma, and help relieve pain and fatigue.
As is true with other chronic diseases, living with scleroderma can place you on a roller coaster of emotions. Here are some suggestions to help you even out the ups and downs:
- Maintain normal daily activities as best you can.
- Pace yourself and be sure to get the rest that you need.
- Stay connected with friends and family.
- Continue to pursue hobbies that you enjoy and are able to do.
The prognosis for scleroderma has improved dramatically over the past 30 years as the ability to detect and treat many of the problems of scleroderma has improved. But if scleroderma makes it difficult for you to do things you enjoy, ask your doctor about ways to get around the obstacles.
Keep in mind that your physical health can have a direct impact on your mental health. Denial, anger and frustration are common with chronic illnesses.
At times, you may need additional tools to deal with your emotions. Professionals, such as therapists or behavior psychologists, may be able to help you put things in perspective. They can also help you develop coping skills, including relaxation techniques.
Joining a support group, where you can share experiences and feelings with other people, is often a good approach. Ask your doctor what support groups are available in your community.
June 14, 2013
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