Scleroderma is an uncommon condition that results in hard, thickened areas of skin and sometimes problems with internal organs and blood vessels.
Scleroderma is caused by the immune system attacking the connective tissue under the skin and around internal organs and blood vessels. This causes scarring and thickening of the tissue in these areas.
There are several different types of scleroderma that can vary in severity. Some types are relatively mild and may eventually improve on their own, while others can lead to severe and life-threatening problems.
There's no cure for scleroderma, but most people with the condition can lead a full, productive life. The symptoms of scleroderma can usually be controlled by a range of different treatments.
There are 2 main types of scleroderma:
Localised scleroderma is the mildest form of the condition. It often affects children, but can occur at any age.
This type just affects the skin, causing 1 or more hard patches to develop. Internal organs aren't affected.
Exactly how the skin is affected depends on the type of localised scleroderma. There are 2 types, called morphoea and linear.
In systemic sclerosis, internal organs can be affected as well as the skin. This type mostly affects women and usually develops between 30 and 50 years of age. Children are rarely affected.
There are 2 types of systemic sclerosis:
Limited cutaneous systemic sclerosis:
Diffuse systemic sclerosis:
In some cases of systemic sclerosis, organs such as the heart, lungs or kidneys are affected. This can cause a range of potentially serious problems, such as shortness of breath, high blood pressure and pulmonary hypertension (high blood pressure in the lungs).
Normally, the body's immune system fights off any germs that infect the body. It responds like this to anything in the body it doesn't recognise, and settles down when the infection has been cleared.
It's thought scleroderma occurs because part of the immune system has become overactive and out of control. This leads to cells in the connective tissue producing too much collagen, causing scarring and thickening (fibrosis) of the tissue.
It's not clear why this happens. Certain genes are thought to be involved, and having a close family member with the condition may increase your risk.
The aim of treatment is to relieve symptoms, prevent the condition getting worse, detect and treat any complications (such as pulmonary hypertension) and help you maintain the use of affected parts of the body.
Common treatments include:
You will also need regular blood pressure checks and other tests to check for any problems with your organs.
If your symptoms are severe, surgery may be needed. For example, hard lumps under the skin may need to be removed, and tightened muscles may need to be loosened.
Newer treatments such as laser therapy and photodynamic therapy are currently being trialled, and may improve the outcome of the condition for many people.
There are many therapies and lifestyle changes that can help reduce the impact of scleroderma on your life.
Regular physiotherapy and stretching exercises may help keep your muscles supple and loosen tight skin.
An occupational therapist can help you adapt to any movement difficulties by suggesting changes to your home and advising you on equipment to make daily life easier.
If you're affected by Raynaud's, you'll need to keep your hands and feet warm in the cold by wearing thick gloves and socks. Read more about treating Raynaud's.
Many people find it helps to read up on the condition and talk to other people who are affected. Scleroderma & Raynaud's UK (SRUK) is dedicated to improving the lives of people affected by scleroderma and Raynaud's.
Women with systemic sclerosis may find it harder to get pregnant and could have a slightly higher risk of miscarriage and giving birth prematurely.
However, if the pregnancy is planned in consultation with a doctor during a period when the condition is stable, there's no reason why a woman with systemic sclerosis cannot have a successful pregnancy.
Scleroderma and Raynaud's UK has more information about systemic sclerosis and pregnancy.