What is Scleroderma?

Types of Scleroderma

There are two main types of scleroderma: localized and systemic. The difference is based on where in the body the symptoms occur. Localized scleroderma only affects the skin, while systemic scleroderma affects many different organs and systems throughout the body.

Approximately 300,000 people in the US suffer from scleroderma. Localized scleroderma is more common in children. Systemic scleroderma can appear at any age, but it usually develops in people between the ages of 25 and 55, and is more common in women.

Scleroderma Symptoms

The name scleroderma is derived from the Greek words for "hard skin," in reference to one of its primary symptoms, the hardening of the skin in patches. There are two types of localized scleroderma. Morphea is a variety of localized scleroderma with hardened, discolored lesions on the skin, while linear scleroderma presents with bands or stripes of thickened skin on the forehead, arms or legs.

Systemic scleroderma has more widespread symptoms, affecting the internal organs as well as the skin. Some symptoms of systemic scleroderma include thickening and formation of scar tissue in the heart, lungs and intestines. High blood pressure is a common symptom of systemic scleroderma.

CREST Syndrome

Individuals with scleroderma often also have other conditions that accompany the disease, which occur together in what is known as CREST syndrome.

• Calcinosis- calcium deposits appear as small white lumps under the skin, primarily on the fingers, knees and elbows; these can be painful and sometimes become infected.
• Reynaud's Syndrome- a condition in which the capillaries of the hands and feet become constricted due to cold or stress, causing the fingers and toes to become numb.
• Esophageal dysfunction- the muscles in the esophagus do not function properly, leading to difficulty swallowing, heartburn and acid reflux (GERD) when stomach acid comes back up into the esophagus.
• Sclerodactyly- hardening or inflammation of the skin between the fingers
• Telangiectasias- red spots or patches on the hands, face or mouth caused by dilated capillaries.

Causes of Scleroderma

The cause of scleroderma is unknown. There is some evidence that it has a genetic factor, as it appears with a slightly higher frequency in families with rheumatic diseases and certain ethnic groups. There is a gene that may lead to an increased susceptibility to the disease, but the condition does not appear to be directly inherited.

Scleroderma Treatment

There is no cure for scleroderma, and the different symptoms are treated according to the individual. Heartburn or GERD may be treated with medication to decrease stomach acid. High blood pressure and pulmonary hypertension are treated with specific blood pressure lowering drugs. Reynaud's syndrome may be relieved by hand warming and medication to dilate blood vessels and improve circulation. Moisturizing lotions may be used to relieve the symptoms of dry, itchy skin. Since scleroderma is an auto-immune disease, immune-suppressing drugs may be prescribed.


Sources:

http://www.medicinenet.com/scleroderma/article.htm
http://www.scleroderma.org/medical/overview.shtm
http://www.mayoclinic.com/health/scleroderma/DS00362
http://www.mayoclinic.com/health/crest-syndrome/DS00580/DSECTION=symptoms