Scleroderma is an uncommon disease that causes hard, thickened areas of skin and may also cause problems with blood vessels and some internal organs in the body.

Scleroderma is a type of connective tissue disease – connective tissue is the mesh of tissue that connects and supports the muscles, joints, organs and skin. People with scleroderma have too much of a protein called collagen in their connective tissue, making it hard and tight. Experts think this happens because the body’s immune system becomes overactive, leading to an excess production of collagen.

Types of scleroderma

There are 2 main types of scleroderma.

Localised scleroderma

This type of scleroderma usually only affects the skin and is considered the milder form of the disease. It results in one or more patches of hard, thickened skin. Sometimes the joints and muscles underneath the areas of hardened skin become stiff, because the skin restricts their movement. Localised scleroderma may be called morphea (patches of hard skin) or linear (long streaks or lines of hard skin).

Diffuse scleroderma (also known as systemic sclerosis)

This type of scleroderma may affect the connective tissue throughout the body. It is often associated with widespread skin changes and can affect the internal organs such as the heart, lungs, digestive tract, muscles, joints and kidneys. There are 2 types of systemic scleroderma: limited (mostly affects the skin; affects about 70% of people with systemic disease) or diffuse (affects the skin and other organs in the body; seen in about 30% of people with systemic scleroderma).

What are the symptoms of localised scleroderma?

  • Someone with the morphea type of localised scleroderma often has localised patches of thickened, hard skin
  • In someone with the linear type of scleroderma, the hard areas of skin are in the shape of long streaks or lines, often starting on the arm, leg, neck or forehead.
  • Skin patches and streaks may appear waxy and may be brown or red in colour
  • Patches may change size over time and can disappear and reappear at different times.

What are the symptoms of systemic sclerosis?

People with systemic sclerosis have similar skin changes to those with localised disease, but these are usually much more widespread over the body.

People may also have:

  • Raynaud’s phenomenon: this affects the blood flow to the limbs, especially the fingers and toes; it can cause the fingers and toes to go white, blue or red in response to cold weather; it is often one of the early symptoms of systemic scleroderma and may affect up to 90% of people with this condition
  • Thickening and tightness of the skin over the fingers and toes (known as sclerodactyly): this may lead to skin ulcers and wasting of the tissue underneath the affected areas
  • Tightness of the skin on the face
  • Small, white lumps of calcium under the skin on the fingers or other parts of the body (known as calcinosis)
  • Stiffness and/or pain in the joints and muscles
  • Red lines or patterns on the skin (known as telangiectasia): caused by widening of the small blood vessels in the skin
  • Problems with swallowing
  • Indigestion (heartburn)
  • Shortness of breath
  • Bloating after eating
  • Weight loss, diarrhoea, constipation.

Causes of scleroderma

Scleroderma develops because of an over-production and build-up of collagen in the body. Exactly why this happens is unknown, but experts think scleroderma is a type of auto-immune disease, where the body’s immune system attacks its own tissue. There may be genetic and environmental factors that trigger the development of scleroderma, and further research is underway to determine how this happens.

Who gets scleroderma?

There are thought to be more than 5,000 Australians with scleroderma. Scleroderma can affect people of all age groups, but often begins between the ages of 25 and 55. The condition affects more women than men.

Diagnosis

There is no one specific test for scleroderma. Scleroderma may be difficult to diagnose in the early stages, as some symptoms are common to some other skin and joint diseases. Different symptoms may appear at different times, and will vary from person to person.

The condition is usually diagnosed by a combination of factors, including the appearance of the skin, blood tests to check for antibodies (known as anti-nuclear antibodies or ANA) and an examination of a sample (biopsy) of affected skin. If the lungs are affected a chest X-ray may also be done.

Treatment of scleroderma

There is no cure for scleroderma, but treatments are available to help relieve the symptoms. The type of treatment will depend on the symptoms each person has. People with systemic scleroderma will often need a range of treatments and may be managed by several medical specialists, depending on what organ systems are affected.

  • Localised scleroderma may be treated with steroid creams and moisturising creams; some people will also need anti-inflammatory and antihistamine medicines.
  • Physiotherapy can help to gently stretch out tight areas of tight skin and improve movement of the underlying joints and muscles.
  • People with Raynaud’s phenomenon or calcium deposits under the skin may need antibiotics if any infection develops, and dressings to cover wounds or ulcers.
  • When the digestive tract is affected, medicines called proton pump inhibitors may be appropriate. Medicines for diarrhoea and constipation may also be required.
  • When scleroderma affects the lungs, treatment with medicines such as corticosteroids can be helpful. Some people will also require medicines that work on the immune system (Immunosuppressants).

What can I do to help manage scleroderma?

Lifestyle changes can help to manage some of the symptoms of scleroderma. For example:

  • If you have Raynaud’s phenomenon, it can be helpful to avoid exposure to cold temperatures; use gloves and warm socks to protect fingers and toes and keep them warm
  • Give up smoking if you smoke
  • Do regular, gentle exercise: talk to a physiotherapist about what is best for you to improve the movement of your skin, joints and muscles
  • Keep your skin clean and well moisturised: try moisturising creams containing sorbolene or lanolin
  • Avoid excessive bathing and hand washing
  • Use a soap alternative when bathing: soap can dry out the skin
  • If you have digestive symptoms such as indigestion, it may be helpful to raise the head of the bed when sleeping
  • Avoid foods that you know trigger any of your digestive symptoms; sometimes eating smaller, more frequent meals can be helpful
  • Take medicines as directed by your doctors
  • Get plenty of rest and try to minimise stress.

Outlook

The outlook for people with scleroderma depends on the type of scleroderma they have, the type of symptoms and the severity of internal organ involvement. Many people have only a few symptoms and can lead normal, active lives. People with localised scleroderma tend to have a milder form of the disease that progresses slowly. People with systemic scleroderma have more widespread symptoms that may develop more quickly. Your doctors can talk to you further about how they think your condition will develop over time, and help you to develop a treatment program to control symptoms.

It can also be helpful to talk to others with the condition. Support groups are available, for example, Scleroderma Australia (http://www.sclerodermaaustralia.com.au) has a range of state-based organisations to assist and support people with scleroderma.

Last Reviewed: 06/07/2016

myDr



References

1. eTG Complete. Inflammatory connective tissue disease. Scleroderma (Amended October 2015). Therapeutic Guidelines Ltd (eTG March 2016 edition). (Accessed June 2016).
2. Scleroderma Australia. Understanding and managing scleroderma. (Fourth edition 2015). http://www.sclerodermaaustralia.com.au/images/publications/Scleroderma_Edition_4.pdf (Accessed June 2016).
3. Arthritis Australia. Scleroderma fact sheet (reviewed May 2015). http://www.arthritisaustralia.com.au/images/stories/documents/info_sheets/2015/Condition%20specific/Scleroderma.pdf (Accessed June 2016).