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What is Scleroderma?

Scleroderma, also referred to as systemic sclerosis, is an autoimmune disorder in which normal tissue is replaced with dense, thick fibrous tissue. The condition is characterized by hardening and tightening of the skin and connective tissue. Although it commonly affects the skin, scleroderma can also affect many other regions of the body including the blood vessels, lungs, gastrointestinal tract, heart, kidneys, muscles, and joints. Scleroderma is a long-term and progressive disease that gradually worsens and can be life-threatening in its most severe form.

Picture of Scleroderma

Causes of Scleroderma

Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. Normally, the immune system assists in defending the body against infection and diseases. However, in patients with scleroderma, the immune system causes other cells to make too much collagen protein. This excessive collagen gets accumulated in the skin and organs, which triggers thickening and hardening of the skin (similar to the process of scarring).

Types of Scleroderma

Scleroderma can be classified based on the location and involvement of internal organs into two types:

  • Localized scleroderma
  • Systemic scleroderma

Localized scleroderma involves skin changes in isolated areas, either as morphea patches or linear scleroderma. Morphea is localized patchy areas of skin that become hard and slightly pigmented and can sometimes cause multiple lesions on the skin. Linear scleroderma is more common in children and features bands of hardened skin localized usually to the arm or leg on one side of the body. Localized scleroderma is the milder form of the disease and will not progress to systemic scleroderma, a more severe form.

Systemic scleroderma affects connective tissues of the skin and also that of the internal organs such as the heart, lungs, kidneys, and digestive tract. The tissues of the affected organs may become hard and fibrous decreasing their efficiency. Systemic Scleroderma is also further categorized as Limited or Diffuse:

  • Limited disease: This is a slower and more benign illness, typically confined to the fingers, hands, and face. It is also referred to as CREST syndrome which stands for Calcinosis, Raynaud phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia.
  • Diffuse disease: This type presents with rapid skin thickening progressing to skin hardening and a high risk of developing fibrous hardening of the internal organs including the lungs, heart, bowels, esophagus, and kidneys.Diffuse disease is a very serious form and can ultimately be fatal.

Symptoms of Scleroderma

The symptoms depend upon the type of scleroderma and which organs are affected such as the skin, heart, lungs, kidneys, and digestive tract. Commonly seen symptoms of scleroderma include:

  • Thick, tight skin on the fingers
  • Sores (ulcers) on the fingertips or toes
  • Hair loss
  • Red spots on the hands and face
  • Pain, stiffness, and swelling of the fingers and joints
  • Calcium deposits in connective tissues
  • Joint pain
  • Raynaud’s phenomenon, i.e., narrowing of blood vessels in the hands or feet
  • Swelling of the esophagus, the tube between your throat and stomach
  • Constipation or diarrhea
  • Shortness of breath
  • Dry cough and wheezing

Diagnosis of Scleroderma

Scleroderma may be diagnosed through the following tests:

  • Blood tests: To assess elevated levels of certain antibodies produced by the immune system
  • Tissue sample: A biopsy of the affected skin is taken for examination in the laboratory.
  • Skin assessment: This involves a thorough evaluation of the skin to assess for clues of involvement of the internal organs.

Depending upon the condition of the patient and the results of the initial evaluation, the doctor may recommend further specific diagnostic tests. These tests may include chest X-ray, lung function test, CAT scanof the lungs, EKG, and echocardiogram.

Treatment for Scleroderma

There is no cure for scleroderma, hence the treatment for scleroderma is aimed at managing the symptoms and preventing irreversible damage to the vital organs. Symptoms vary in different individuals so treatment is customized to each patient. Treatment includes non-steroidal anti-inflammatory drugs, immune-suppressants, vasodilators, corticosteroids, and blood pressure medications to reduce the ill effects of the immune response. Additional medications are also given for controlling the other symptoms; however, there is no drug for skin thickening and hardening.

The skin lesions associated with scleroderma can be camouflaged through cosmetic procedures such as exposure to ultraviolet light or laser surgery.

Physical or occupational therapy may be recommended to manage pain and improve your strength and mobility.

In some cases, such as finger ulcers in severe Raynaud’s disease, surgical amputation may be recommended. Similarly, patients with severe pulmonary hypertension may require lung transplants.

Patients with a mild form of scleroderma may not require any treatment.

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