September 26, 2011

Scleroderma is a group of diseases that involve abnormal growth of connective tissue, which is the “glue” made of proteins and cells that holds the cells of the body together and gives the skin and organs their shape. In some people, scleroderma affects the skin, causing it to become tight and hard. In others, it can affect the blood vessels and internal organs such as the lungs and kidneys. Scleroderma is not contagious and is not considered malignant. It is a common disease, with approximately 40,000-165,000 Americans having the disease. Women suffer from scleroderma three times more often than men.

Scleroderma can be divided into two classes: localized scleroderma and systemic sclerosis. Localized scler-oderma is limited to the skin and does not affect internal organs. Localized scleroderma is more common in children. Two common types of localized scleroderma are morphea and linear scleroderma. Morphea usually leads to patches of skin that are thick and firm, and usually fade away in 3-5 years. Linear scleroderma produces a band of thick skin that usually runs down an arm or leg. Localized scleroderma does not develop into the systemic form of the disease.

Systemic scleroderma or systemic sclerosis involves the skin and other organ systems. Typically this develops rapidly with skin thickening over much of the body usually in a symmetrical fashion. Internal organs such as the lungs and kidneys are often involved. People with skin thickening commonly complain of joint pain, fatigue, dryness of the eyes or mouth, swelling of the hands and feet, and weight loss. Patients may also complain of Raynaud’s phenomenon, which is a condition in which the small blood vessels of the hands contract when they become cold, which causes the fingertips to turn blue, red, or white. Fortunately, less than one third of patients with systemic scleroderma develop severe internal organ problems.

The cause of scleroderma is unknown. Many believe scleroderma to be an autoimmune disease in which one’s own immune system attacks the body. Scleroderma is diagnosed mainly by a medical history and physical exam. The physician may perform lab tests to help to confirm the diagnosis of scleroderma. The lab tests may include looking for two antibodies that are commonly found in the blood of patients with sclero-derma (anti-Scl-70 and anticentromere antibodies). These tests may not be positive in all individuals with scleroderma and a skin biopsy may be required to make the diagnosis.

At the present time, there is no treatment that reverses or halts the development of abnormal connective tissue in scleroderma. Treatment of scleroderma consists mainly of relieving symptoms of the disease. Because of the wide variety of symptoms, patients may have many different health professionals involved in their treatment including a dermatologist, a rheumatol-ogist, physical therapists, a nephrologist, and many others.

See Also: Autoimmune disorders, Raynaud’s phenomenon, Skin care, Skin disorders

Suggested Reading

  • Freedberg, I., Eisen, A., Wolff, K., Austen, K. F., Goldsmith, L., Katz, S., et al. (1999). Fitzpatrick’s dermatology in general medicine (6th ed.). New York: McGraw-Hill.
  • Odom, R. B., James, W. D., & Berger, T. G. (Eds.). (2000). Andrews’ diseases of the skin (9th ed.). Philadelphia: W.B. Saunders.


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