Scleroderma

Autoimmune Disorder

What is Scleroderma?

Scleroderma is a disease that involves the buildup of scar-like tissue in the skin. It also damages the cells the line the walls of the small arteries.

Signs and Symptoms

  • Swelling of the hands and feet
  • Red spots on the skin
  • Excessive calcium deposition in the skin
  • Joint contractures
  • Weight loss
  • Hair loss
  • Tight, mask-like facial skin
  • Ulcerations on the fingertips and toes
  • Pain and stiffness in the joints
  • Persistent cough
  • Shortness of breath
  • Heartburn
  • Difficulty swallowing
  • Digestive and gastrointestinal problems
  • Constipation
  • Fatigue

Pathogenesis

The exact cause of scleroderma is unknown. Although rarely scleroderma can run in families, most cases do not show any family history of the disease.

Diagnosis

  • Blood tests
  • Pulmonary function tests
  • Electrocardiogram
  • Echocardiogram
  • Gastrointestinal tests
  • Kidney funtion

Management

There is currently no cure for scleroderma. Instead, treatment is directed at controlling and managing the symptoms.


  • Skin treatments
  • Digestive remedies
  • Treatment of lung disease
  • Exercise
  • Joint protection
  • Skin protection
  • Diet
  • Dental care
  • Stress Management

Prognosis

Localized scleroderma nearly always carries a good prognosis and a normal lifespan. Even localized scleroderma, however, can cause some severe effects in children, including impaired growth, limb imbalance, and problems with flexing and beding muscle. Five-year survival among these patients has remained steady at around 90%.

Epidemiology

Approximately 250 persons per million American adults are affected by scleroderma. It usually develops between the ages of 35 and 55, although a pediatric form also occurs. Scleroderma is four times more common in women than it is in men.