Scleroderma

What is scleroderma?

Scleroderma is an autoimmune condition of the connective tissue under the skin and around organs and blood vessels, that causes the immune system to attack the body. This leaves scarring and causes the tissue to thicken.

Different types of scleroderma vary in severity – some can self-resolve while others can cause life-threatening problems. There’s currently no cure, but most people will live full, productive lives.

What are the different types of scleroderma?

The main types of scleroderma are:

Localised scleroderma

Localised scleroderma mainly affects your skin and is the mildest form. There are two types of localised scleroderma:

1. Morphoea scleroderma – This only affects your skin and is the mildest type of scleroderma. It can resolve after a few years without treatment. It causes shiny patches on your skin that are usually itchy.

2. Linear scleroderma – This also affects your skin, but in rare cases can affect the underlying muscle and bone which can stop your limb growing. It appears as thickened lines on your skin.

Systemic sclerosis

Systemic sclerosis affects your skin and can also affect blood vessels and internal organs. Women are affected more frequently, and it usually develops between the ages of 30 and 50. There are 2 types of systemic sclerosis:

1. Limited cutaneous systemic sclerosis – This affects the skin of your hands and face, as well as a limited effect on the organs and blood supply to your hands and feet. This usually worsens with age but can be controlled with treatment. This is the milder of the 2 forms.

2. Diffuse systemic sclerosis – This is more likely to affect the internal organs and the skin of the whole body. Symptoms can worsen quickly over a few years before settling and occasionally improving again.

What are the symptoms of scleroderma?

Symptoms vary person to person and depending on the type of scleroderma.

Skin manifestations are usually the first sign of scleroderma and include:

• Tightening of your skin that can affect movement, usually on your hands, face and fingertips

• Discoloured patches that can look like bruises. They can be anywhere and are often shiny, hairless and itchy.

• Thickening of the skin in lines on the face, scalp, arms or legs

• Hard lumps under your skin

• Tiny blood vessels beneath your skin

It’s common for people with scleroderma to have Raynaud’s, a condition that affects blood supply to the hands and fingers resulting in painful, pale hands that get worse when it’s cold. This can be an early sign of systemic sclerosis.

When your organs are affected, it can cause a range of potentially serious problems affecting your heart, lungs, kidneys and digestive tract. Swallowing difficulties and heartburn can also be caused by systemic sclerosis.

Scleroderma & Raynaud’s UK have more information on organ involvement.

How common is scleroderma?

Scleroderma is a rare condition – there are around 19,000 people in the UK with scleroderma and 2.5 million worldwide.

What causes scleroderma?

The cause of scleroderma is not fully understood, but it’s an autoimmune disease, meaning your body’s immune system attacks your healthy body tissue. This leaves scarring which causes tightening of the affected tissue resulting in the symptoms you will experience.

How is scleroderma diagnosed?

Scleroderma is hard to diagnose because it’s rare, and it’s common for doctors to never have seen a case before. If a doctor suspects you have scleroderma they will refer you to a specialist team. There’s no specific test to diagnose scleroderma so instead, lots of different information is used by a specialist team to give you a diagnosis.

How is scleroderma treated?

Scleroderma can’t be cured, so treatments are to help control symptoms. Steroids and immunological drugs aim to ‘turn down’ the immune system to stop it attacking the body.

Common treatments include:

• Medicines to improve circulation

• Intense moisturiser usage to soften skin

• Medications for heartburn, blood pressure and pain

Once diagnosed, you’ll need regular blood pressure monitoring and other tests to check for any problems with your organs.

If your symptoms are severe, you may need surgery. Examples include loosening of tight muscles or removal of hard painful lumps under the skin.

How long does scleroderma last?

Duration depends on the type of scleroderma and varies between people. Localised scleroderma can last a few years and resolve on its own whereas systemic sclerosis is long-term and can last your lifetime.

When should I seek help?

If you think you have scleroderma, speak to a doctor who will be able to assess your symptoms and refer you for specialist assessment if they think you need it.

If you’ve already been diagnosed with scleroderma, you may be at risk of having complications, so seek help if:

• Your blood pressure is suddenly increasing

• You become short of breath

• You’re on immunosuppressive treatments such as steroids and you get an infection