Victoria, BC

Tillicum Medical Clinic

Scleroderma

Scleroderma is a rare and chronic condition that can affect many parts of your body including the skin, joints, blood vessels and internal organs. The word "scleroderma" means hard skin and can include skin changes and various other symptoms. Scleroderma can be either limited, affecting mainly the hands and face, or diffused, affecting the arms, legs and trunk.

The cause of scleroderma is unknown. The immune system may cause excess amounts of a protein called collagen to deposit in the skin and organs and cause thickening and hardening of the skin.

The condition affects women three to five times more often than men. It usually starts between the ages of 30 to 50, but also can affect children and the elderly.

Scleroderma can affect everyone differently. The following are some of the general symptoms associated with the condition.

Skin changes, including:

  • Hardening or thickening of your skin, particularly on your hands, arms and face, and occasionally on your trunk and legs
  • Loss of hair over the affected area
  • Change in skin color
  • Ulcers or sores on your fingers

Swelling or puffiness in your hands or feet, often occurring in the morning.

Sclerodactyly means shortening of the skin of the fingers and toes. This usually occurs after the initial swelling goes away. You may experience:

  • Skin on your fingers and toes becoming hard and shiny
  • Difficulty bending your fingers

Raynaud's Phenomenon, a condition associated with poor blood flow to fingers and toes. Blood flow decreases because blood vessels in these areas become narrow for a short time in response to cold or emotional stress. Raynaud's Phenomenon may cause:

  • Your fingers, toes, tips of your ears, nose or tongue to be very sensitive to cold
  • Your fingers to turn bluish or very pale
  • Your fingers to feel tingly, numb or cold

Telangiectasia, which occurs when tiny blood vessels become dilated and show through your skin. Small reddish spots may appear on your fingers, palms, face, lips and tongue. These spots are harmless and can be hidden with cosmetics.

Calcinosis, which occurs when small white calcium lumps form in or under the skin. It is caused by scleroderma and not by too much calcium in your diet. The lumps occasionally break through the skin and leak a chalky, white material. If injured, they may become infected.

Arthritis and muscle weakness, including:

  • Pain, stiffness and swelling
  • General fatigue
  • Muscle weakness, often in your upper arms or thighs

Digestive problems including poor function of your esophagus and bowels. Symptoms may include:

  • Difficulty swallowing
  • Heartburn
  • Bloating
  • Nausea or vomiting
  • Weight loss
  • Diarrhea or constipation

If the heart and lungs are affected, you may experience:

  • Shortness of breath
  • Persistent cough
  • Chest pain

Kidney problems, such as kidney failure and high blood pressure. Symptoms may include:

  • Fatigue
  • Severe headache
  • Shortness of breath
  • Trouble seeing
  • Mental confusion

If you are suffering from any of these symptoms, consult a physician as soon as you can.

There is no direct cure for scleroderma. Because the exact cause is unknown, any treatment is patient-specific and aimed at improving symptoms of the disease. For example, patients who experience Raynaud's phenomenon may be treated with agents to increase blood flow to the fingers. Fibrosis of the skin has been treated with varying degrees of success with agents such as d-penicillamine, colchicine, PUVA, Relaxin, and cyclosporine. Because scleroderma is an autoimmune disease, one of the major pillars of treatment involves the use of immunosuppressive agents.

Individuals with limited scleroderma have a relatively positive outlook. They will usually die from another disease, not the scleroderma. Those with very widespread skin and organ involvement (systemic) have a negative prognosis. More women have scleroderma, but the disease kills more men. Following diagnosis, two-thirds of patients live at least 11 years. The higher the patient's age at diagnosis, the more likely they are to die from the disease.