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Scleroderma

What is scleroderma?

Scleroderma is a chronic but rare autoimmune disease where normal tissues are replaced with thick tissue with extra collagen. Under normal circumstances, the immune system defends the body against disease and infection. In children with scleroderma, the immune system triggers other cells to produce too much collagen, which will be deposited in the skin and organs. This causes hardening and thickening of the skin. 

Even though scleroderma often affects the skin, it may also affect other parts of the body such as the gastrointestinal tract, kidneys, lungs, blood vessels, heart, muscles and joints. Severe scleroderma can be life threatening.

What are the symptoms of scleroderma?

Besides the most common symptom being the thickening of the skin, scleroderma may cause other symptoms such as:

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

Early symptoms of scleroderma include fingers that are highly sensitive to the cold and change colour with cold or emotional stress. Fingers may also be stiff and puffy. These symptoms occur due to the blood vessels narrowing due to spasms. Excess collagen may also damage blood vessels.

There are two different types of scleroderma, namely:

• Localised scleroderma - This is a type of scleroderma that affects only the skin and not major organs.
• Systemic scleroderma – This is a type of scleroderma that affects the skin and may affect the tissues under the skin, including blood vessels and major organs like the gastrointestinal tract, heart, lungs or kidneys.

What are the treatment options for scleroderma?

Scleroderma may have many symptoms; a combination of approaches is often required to treat and manage the disease effectively. Dr Ambaram may recommend the following:

• Skin treatments - For localised scleroderma, topical medications may be prescribed. Disease-modifying agents like Methotrexate or Mycophenolate Mofetil may also be prescribed to treat localised scleroderma.
• Digestive treatments - A variety of medications that may be prescribed to help treat heartburn and other digestive difficulties. These medications include antacids, proton pump inhibitors and H2receptor blockers.
• Lung disease treatment - Patients with scleroderma who have rapidly worsening scarring of the lung tissue (pulmonary fibrosis), cyclophosphamide may be prescribed.