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What is Scleroderma?

Scleroderma, also known as systemic sclerosis, is a rare and devastating autoimmune disease in which the body attacks its own tissues, causing an overproduction of collagen. Collagen is a fiber-like protein which holds all of the structures in the body together. Individuals with scleroderma often suffer fatigue, lose appetite and weight, and have joint swelling and/or pain. Severe forms of the disease typically cause significant thickening and stiffening of the skin. Internally, it can damage organs such as the heart, lungs, and kidneys.

Because scleroderma can be hard to diagnose and it overlaps with or resembles other conditions, scientists can only estimate how many cases actually exist. Current estimates are that 75,000 to 100,000 Americans have the disease, of which the majority are women in their child-bearing years. In people with severe forms, mortality rates are high — almost 50% die within five years of diagnosis.

Types of scleroderma

Scleroderma can affect the internal organs of the body, large areas of skin, or both. The disease can be classified into two groups:

  • Localized — only the skin is affected
  • Systemic — can affect the whole body (skin, tissues, blood vessels, and internal organs

Systemic scleroderma, also called systemic sclerosis (SSc), can be further classified as either limited or diffuse:

  • Limited scleroderma — comes on gradually and affects the skin only in certain areas (hands, face, lower arms, and legs); skin thickening may take years to develop
  • Diffuse scleroderma — skin thickening usually occurs quickly and over much of the body (hands, face, upper arms, upper legs, chest, and stomach); connective tissue and internal organs (heart, lungs, and kidneys) are often affected.