Scleroderma (sklere-o-DUR-muh) is a group of rare, progressive diseases that involve the hardening and tightening of the skin and connective tissues — the fibers that provide the framework and support for your body. Localized scleroderma affects only the skin. Systemic scleroderma also harms internal organs, such as the heart, lungs, kidneys and digestive tract. Scientists estimate that about 250 people per million have some form of scleroderma. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease. Scleroderma isn't considered contagious, but it can greatly affect self-esteem and the ability to accomplish everyday tasks.

Complications of Scleroderma

Scleroderma complications can range from mild to severe. Some may even become life-threatening.

Raynaud's complications

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 ulcers in the flesh. In some cases, gangrene and amputation may follow.

Lung complications

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).

Kidney complications

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 complications

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).

Dental complications

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 decay increases even more. In addition, acid reflux can destroy tooth enamel, and changes in gum tissue may cause your teeth to become loose or even fall out.

Sexual complications

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.

Causes of Scleroderma

• 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.

Signs & Symptoms of Scleroderma

Scleroderma symptoms vary, depending on which organ systems are involved. Diagnosis can be difficult because some of the early symptoms are common in the general population, and aren't always associated with scleroderma. The most prevalent signs and symptoms of scleroderma include:

• Raynaud's phenomenon. An exaggerated response to cold temperatures or emotional distress, this condition constricts the small blood vessels in the hands and feet and causes numbness, pain or color changes in the fingers or toes.
• Gastroesophageal reflux disease (GERD). In addition to acid reflux, which can damage the section of esophagus nearest your stomach, you may also have problems absorbing nutrients if your intestinal muscles aren't moving food properly through your intestines.
• Skin changes. These changes may include swollen fingers and hands; thickened patches of skin, particularly on the fingers; and tight skin around the hands, face or mouth. Skin can appear shiny because it's so tight, and movement of the affected area may be restricted.

Localized scleroderma

The two main varieties of localized scleroderma, which affects only the skin, are distinguished by very distinctive signs and symptoms:

• Morphea. This variety features oval-shaped thickened patches of skin that are white in the middle, with a purple border.
• Linear scleroderma. This variety, which is more common in children, features bands or streaks of hardened skin on one or both arms or legs, or on the forehead.

Systemic scleroderma

This type of scleroderma, also called systemic sclerosis, affects not only your skin but also your blood vessels and internal organs. Subcategories are defined by what portions of your body are affected. One variation is known as limited scleroderma, or CREST syndrome.

Diagnosis of Scleroderma

Your doctor may also conduct the following tests:

• Blood tests. People with scleroderma usually have elevated blood levels of certain antibodies produced by the immune system.
• Tissue sample. Your doctor may remove a small tissue sample (biopsy) of your affected skin to be examined in the laboratory for abnormalities.

Your doctor may recommend other diagnostic tests to identify any lung, heart or gastrointestinal complications accompanying scleroderma.

Treatments of Scleroderma

Scleroderma has no known cure — no drug will stop the overproduction of collagen. But the localized variety of scleroderma sometimes resolves on its own. And a variety of medications can help control the symptoms of scleroderma or help prevent complications.

Medications

• Dilating the blood vessels. Blood pressure medications that dilate blood vessels may help prevent lung and kidney problems and may help treat Raynaud's disease.
• Suppressing the immune system. Drugs that suppress the immune system, such as those taken after organ transplants, may help reduce scleroderma symptoms.

Therapy

• Physical or occupational therapy. Therapists can help you manage pain, improve your strength and mobility, and work on performing essential daily tasks to maintain your independence.
• Cosmetic procedures. The appearance of skin lesions associated with scleroderma may be helped by exposure to ultraviolet light. Laser surgery also may help camouflage or eliminate these lesions.

Surgery

• Amputation. If finger ulcers caused by severe Raynaud's disease 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.