Scleroderma, which means “hard skin,” affects about 300,000 Americans. It is not contagious, infectious or cancerous. Scleroderma refers to two conditions - localized scleroderma and systemic sclerosis.

Localized scleroderma

This condition mainly affects the skin, but also can affect tissues beneath the skin including fat, connective tissue, muscle and bone. Localized scleroderma is more common in children. In adults, it affects women more often than men, and can appear between the ages of 20 and 50.

There are two types of localized scleroderma:

  • morphea, which is characterized by one or more hard, oval-shaped, whitish or darkened patches of skin.
  • linear, in which lines or streaks of thickened skin form over an area of the body, such as an arm, leg or the head, and may cause deep-tissue injury (ulcers on the skin).

Systemic Sclerosis

This condition can affect many parts of the body, such as the skin, blood vessels, digestive system, heart, lungs, kidneys, muscles and joints. Thirty percent of people with scleroderma have the systemic form. Systemic sclerosis may be classified as either limited or diffuse.

  • Limited scleroderma affects the skin on the face, fingers and hands, and lower arms and legs. For many people, the first symptoms of systemic sclerosis are Raynaud’s phenomenon and puffy fingers, which can begin several years before other symptoms. If internal organs are involved, it tends to be mild. However, some people experience severe Raynaud’s phenomenon, gastrointestinal problems or serious effects on the lungs. Some patients with limited scleroderma also have CREST syndrome. CREST is an acronym using the first letter of five conditions: calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia. These conditions are explained on page 2.

  • Skin thickening from diffuse scleroderma is widespread. It may affect any part of the body, especially the hands, arms, thighs, chest, abdomen and face. Itching, decreased flexibility and pain can also occur. Diffuse scleroderma may affect the blood vessels, heart, joints, muscles, esophagus, intestines and lungs. The severity of internal organ involvement varies. Kidney problems may lead to high blood pressure and, if untreated, kidney failure. Lung damage is the leading cause of death with this condition. In rare cases, systemic sclerosis may involve internal organs but not the skin. This condition is known as systemic sclerosis sine scleroderma.