Scleroderma - The Basic Facts

An Autoimmune Disease of Connective Tissue

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What Is Scleroderma?

Scleroderma is an autoimmune, connective tissue disease. Scleroderma is not a single disease, but rather a symptom of a group of diseases complicated by the abnormal growth of connective tissue which supports the skin and internal organs. Some types of scleroderma are limited to skin thickening and tightening. Other types involve blood vessels and internal organs.

What Are the Types of Scleroderma?

There are two broad types of scleroderma - localized and generalized (also referred to as systemic sclerosis).

Localized Scleroderma

Localized scleroderma primarily affects the skin and is further divided into morphea (hard, oval patches on the skin) and linear (a distinct line of thickened and abnormally colored skin often affecting arms, legs, or the forehead).

Systemic Sclerosis

Systemic sclerosis is divided into limited scleroderma (gradual onset, restricted to certain areas of the body, can affect internal organs later) and diffuse scleroderma (sudden onset, thickening covers larger area and can affect internal organs). Sine scleroderma, which some consider a third category of systemic sclerosis, does not affect the skin.

What Is CREST?

People with systemic sclerosis may have symptoms which fit a characteristic pattern, referred to as CREST:

  • Calcinosis (calcium deposits in connective tissue)
  • Raynaud's phenomenon (small blood vessels to fingers, toes, nose, and ears constrict in response to cold or anxiety)
  • Esophageal dysfunction (muscle in lower part of esophagus does not function well)
  • Sclerodactyly (thick, tight, shiny skin on fingers or toes)
  • Telangiectasias (small, red spots on hands and face)

    Scleroderma Diagnosis

    No single test serves as a definitive diagnosis for scleroderma. A combination of findings from physical examination, blood tests, and tests which can evaluate problems with organs of the body help diagnose scleroderma. Most people with scleroderma have positive tests for antinuclear antibodies.

    Scleroderma Treatment

    There is no cure for scleroderma. No drug can yet stop skin thickening associated with scleroderma. Medications are prescribed to control symptoms which may include:

    • Raynaud's phenomenon
    • heartburn
    • high blood pressure
    • interstitial lung disease
    • pulmonary hypertension
    • arthritis
    • muscle weakness
    • digestive problems
    • kidney problems

    Scleroderma Prevalence

    Scleroderma affects 75,000 - 100,000 people in the United States. Women between 30 and 50 years old account for 75% of scleroderma cases. Men and children also can develop scleroderma. According to the American College of Rheumatology, 12 to 20 new cases of scleroderma per million are diagnosed in the United States each year.


    Scleroderma, Arthritis Foundation

    Scleroderma (Systemic Sclerosis), American College of Rheumatology

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