Scleroderma

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Systemic sclerosis (scleroderma) is a rare, chronic disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities.

• Swelling of the fingers, intermittent coolness and blue discoloration of the fingers, joints freezing in permanent (usually flexed) positions (contractures), and damage to the gastrointestinal system, lungs, heart, or kidneys may develop
• People often have antibodies in the blood characteristic of an autoimmune disorder
• No treatment changes the course of the disorder
• Symptoms and organ dysfunction are treated.

The cause of systemic sclerosis is not known. The disorder is 4 times more common among women than men and is rare among children. Symptoms of systemic sclerosis may occur as part of mixed connective tissue disease, and some people with mixed connective tissue disease develop severe systemic sclerosis. Systemic sclerosis can occur in limited forms, for example, sometimes affecting just the skin or mainly only certain parts of the skin or as CREST syndrome. However, systemic sclerosis often causes damage that is widespread throughout the body (called diffuse or generalized systemic sclerosis).

Symptoms

The usual initial symptom of systemic sclerosis is swelling, then thickening and tightening of the skin at the ends of the fingers. Raynaud's syndrome, in which the fingers suddenly and temporarily become very pale and tingle or become numb, painful, or both in response to cold or emotional upset, is also common. Fingers may become bluish. Heartburn, difficulty in swallowing, and shortness of breath are occasionally the first symptoms of systemic sclerosis. Aches and pains in several joints often accompany early symptoms. Sometimes inflammation of the muscles (polymyositis), with its accompanying muscle pain and weakness, develops.

Skin Changes: Systemic sclerosis can damage large areas of skin or only the fingers (sclerodactyly). Sometimes systemic sclerosis tends to stay restricted to the skin of the hands. Other times, the disorder progresses. The skin becomes more widely taut, shiny, and darker than usual. The skin on the face tightens, sometimes resulting in an inability to change facial expressions. Sometimes dilated blood vessels (telangiectasia often referred to as spider veins) can appear on the fingers, chest, face, lips, and tongue, and bumps composed of calcium can develop on the fingers, on other bony areas, or at the joints. Sores can develop on the fingertips and knuckles.

Joint Changes: Sometimes, a grating sound can be felt or heard as inflamed tissues move over each other, particularly at and below the knees and at the elbows and wrists. The fingers, wrists, and elbows may become stuck (forming a contracture) in flexed positions because of scarring in the skin.

Gastrointestinal System Changes: Scarring commonly damages the lower end of the esophagus (the tube connecting the mouth and stomach). The damaged esophagus can no longer propel food to the stomach efficiently. Swallowing difficulties and heartburn eventually develop in many people who have systemic sclerosis. Abnormal cell growth in the esophagus (Barrett's esophagus occurs in about 33% of the people, increasing their risk of esophageal blockage (stricture) due to a fibrous band or their risk of esophageal cancer. Damage to the intestines can interfere with food absorption (malabsorption) and cause weight loss.

Lung and Heart Changes: Systemic sclerosis can cause scar tissue to accumulate in the lungs, resulting in abnormal shortness of breath during exercise. The blood vessels that supply the lungs can be affected (their walls thicken), so they cannot carry as much blood. Therefore blood pressure within the arteries that supply the lungs can increase (a condition called pulmonary hypertension. Systemic sclerosis can also cause several life-threatening heart abnormalities, including heart failure and abnormal rhythms.

Kidney Changes: Severe kidney disease can result from systemic sclerosis. The first symptom of kidney damage may be an abrupt, progressive rise in blood pressure. High blood pressure is an ominous sign, although treatment usually controls it.

Treatment

Individual treatment of scleroderma may vary depending upon the patient and their desire to receive tradition treatment, alternative treatment or integrative treatment for the disease.  It is vital that persons diagnosed with, or suspected of having scleroderma consult with their health care provider to assure proper evaluation, treatment and interpretation of information contained on this site.

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