Scleroderma

Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are three major forms of the disease:

  • Causes

    Overproduction of collagen and other connective tissue proteins are the main features of scleroderma. It is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production.

  • Definition

    Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are three major forms of the disease:

    • Localized scleroderma (can be morphea or linea)—Usually affects only the skin in isolated parts of the body. This form is less serious.
    • Systemic sclerosis—Affects widespread areas of skin and/or internal organs, most often the lungs. Certain categories of this form of scleroderma are more serious and can be fatal.
    • Overlap syndrome—May involve features of scleroderma and features of other autoimmune syndromes.

  • Diagnosis

    The doctor will ask about your symptoms and medical history. A physical exam will be done. Diagnosis is based on changes in the skin.

    Tests may include:

    • Blood tests
    • Esophagus motility study
    • Biopsies
      of skin and other tissues

    Imaging tests take pictures of internal body structures. These may include:

    • X-ray
    • MRI
    • CT scan

  • Prevention

    There are no current guidelines to prevent scleroderma.

  • Risk Factors

    Scleroderma is more common in women. The morphea type of scleroderma usually strikes people around 20-40 years old. Linear scleroderma often occurs in children. Systemic scleroderma is more likely to occur in people aged 30-50 years old.

    Other factors that may increase your chance of developing scleroderma include:

    • Family members with autoimmune disorders
    • Occupational chemical exposure, such as silica, ketones, or welding fumes

  • Symptoms

  • Treatment

    There is no treatment to cure scleroderma. Treatments are aimed at relieving symptoms in various systems.