Scleroderma

Scleroderma

Scleroderma is a chronic autoimmune disease that causes hardening of the skin. It is not contagious.

Causes and risk factors

Scleroderma can occur in both adults and children but is more often seen in females in the age group of 25 to 50 years. The exact cause of scleroderma is not known. An abnormal immune response of the body against its own tissue is known to cause scleroderma. It is not genetic and cannot be passed from parents to children although some genes are known to increase the risk for the disease.

Types

Scleroderma is of two main types:

Localized: It affects mainly the skin of the face and hands and may cause streaks of thick skin (linear scleroderma) or discolored patches (morphea). However it may also spread to muscles, joints and bones and can cause serious complications.

Systemic: It affects not only skin but also muscles, joints, bones and even other organs such as heart, lungs and kidneys. Thus it is a more serious form of the disease than localized scleroderma and can even be fatal.

Signs and Symptoms

The signs and symptoms include:

  • Reynaud’s phenomenon: This is a characteristic sign of scleroderma and is present in most of the patients. It occurs from a temporary lack of blood circulation to the fingers and toes in response to cold and hot temperatures. This lack of blood supply causes color changes in the fingers and toes to white, blue and then red
  • Skin changes: Scleroderma causes hardness, tightening and swelling of the skin. This may lead to curling and loss of function of the fingers. It sometimes even changes the physical appearance of the face significantly
  • Scleroderma also causes sores on the fingers and toes
  • Telangiectasia, flat red marks on the face and hand, may be present. These are formed from dilated blood vessels on the skin
  • Swelling, pain and stiffness of joints
  • Problems of digestive tract including heartburn, constipation, bloating, and difficulty in swallowing, diarrhea and fecal incontinence
  • Dry cough and shortness of breath with wheezing
  • Effect on kidneys can lead to high blood pressure
  • Calcium deposits on skin and other connective tissue

Diagnosis

To diagnose scleroderma the doctor will ask your medical history and will do a physical examination to look for some specific signs of the disease. The doctor will also order some tests that may include blood tests, chest X-ray, Echocardiogram, urine analysis, CT scan of the lungs and skin biopsy. Blood tests may include antinuclear antibody panel, antibody testing, Erythrocyte Sedimentation Rate (ESR) and rheumatoid factor test.

Treatment

The treatment for Scleroderma is aimed at managing the symptoms and preventing irreversible damage to the vital organs. Symptoms vary in different individuals and so treatment is customized to each patient. Treatment includes non-steroidal anti-inflammatory drugs, immune-suppressants and corticosteroids to reduce the improper immune response. Additional medications are also given for controlling the other symptoms; however, there is no drug for skin thickening and hardening. Apart from treatment, continuous support of friends and family is critical for improving the quality of life of the patient.

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Scleroderma