What Is Scleroderma?

Learn the basics about this autoimmune disease of the connective tissue

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Scleroderma is an autoimmune, connective tissue disease. It 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.

What Causes Scleroderma?

The reason the body attacks its own cells in this autoimmune disease is not known. Researchers do not believe that scleroderma is inherited, either, but individuals from families with a history of it or other rheumatic diseases may be more prone to developing it.

In some cases scleroderma has been linked being exposed to certain chemicals or pollutants—in other words, environmental factors.

Prevalence

Scleroderma affects 75,000 to 100,000 people in the United States. According to the American College of Rheumatology, 12 to 20 new cases of scleroderma per million are diagnosed in the United States each year. About one-third of these individuals have systemic sclerosis, which affect the entire body.

Who Gets It?

Women between 30 and 50 years old account for 75 percent of scleroderma cases, but men and children of any age group can also develop it. The condition usually develops between the ages of 25 to 55.

Types of Scleroderma

There are two broad types of scleroderma: localized and generalized (also referred to as systemic sclerosis). Localized is limited to skin thickening and tightening, while generalized types involve blood vessels and internal organs.

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.

Common Symptoms: 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)

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. A proper diagnosis is needed to pinpoint a right treatment.

Treatment Options

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

  • pulmonary hypertension
  • arthritis
  • muscle weakness
  • digestive problems
  • kidney problems

A Word From Verywell

If you suspect or have been diagnosed with scleroderma or systemic sclerosis, getting a proper diagnosis and learning more about your condition can help you better manage it. There is yet no cure for the disease, but there are effective strategies for living a better life.

Sources:

Scleroderma, Arthritis Foundation

Scleroderma (Systemic Sclerosis), American College of Rheumatology

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