Scleroderma is the name for a range of conditions that affect your immune system (autoimmune conditions) and can cause hard, thickened areas of skin and sometimes problems with muscles, bones, internal organs and blood vessels.
The two main types of scleroderma are:
There are also different types of localised scleroderma and systemic sclerosis. Some types may eventually improve on their own, while others can lead to severe and life-threatening problems.
Although there's no cure for scleroderma, symptoms can usually be managed by a range of different treatments.
Localised scleroderma (morphoea) can affect both children and adults.
It mostly affects the skin, causing 1 or more hard patches to develop. But it can also affect tissues such as muscle and bone under the skin.
Exactly how the skin is affected depends on the type of localised scleroderma. There are 2 main types, called plaque and linear morphoea.
Plaque morphoea:
Linear morphoea:
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.
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 may also be available.
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.
It's important to eat healthily, exercise regularly and stop smoking (if you smoke) to keep blood pressure under control and improve your circulation. Read more about controlling high blood pressure.
Many people find it helps to read up on the condition and talk to other people who are affected. Scleroderma & Raynaud's UK (SRUK) work to improve 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.