Page update: 14 June 2021

Understanding Scleroderma

Scleroderma is a rare, chronic disease of the immune system, blood vessels and connective tissue. There are 2.5 million people worldwide who are living with scleroderma, including around 19,000 within the UK.

'Scleroderma' comes from the Greek, 'sclero,' meaning hard, and 'derma', meaning skin. It is an autoimmune condition, meaning that the immune system becomes overactive and attacks healthy tissue within the body. Hardening of the skin can be one of the first noticeable symptoms, as the body produces too much collagen. This excess of collagen can affect the skin, joints, tendons and internal organs. It causes scarring to develop that stops the affected parts of the body from functioning normally.

Symptoms

Everyone’s experience of scleroderma is different. It depends on what type you have, how severe it is, and what parts of your body are affected.

Skin changes
For most people, scleroderma causes problems with the skin. These symptoms can include:
• patches of thick, hard skin that may become discoloured
• itching
• tight skin that makes it harder to move your joints
• hard lumps under your skin (calcinosis)
• tiny blood vessels (spider veins) appearing just beneath your skin, called telangiectasia

You can develop painful sores on your fingers and toes, called digital ulcers. These must be treated right away to protect the tissue and prevent an infection.

Raynaud’s and scleroderma

Alongside scleroderma, it’s common to have a condition called Raynaud’s. People with Raynaud’s are sensitive to temperature changes or cold, and this can set off tingling, discomfort or pain, usually in the hands and feet. Your fingers or toes might turn from white, to blue, to red. Stress and anxiety are sometimes a trigger.

Types of scleroderma

There are two main types of scleroderma:

'localised scleroderma' (also known as morphoea). This only affects the skin, causing one or more patches of hardened skin to develop on the body.

'systemic sclerosis', that can also affect other organs and systems of the body.

Localised scleroderma

Morphoea is the name given to localised patches of hardened skin that appear smooth and shiny. They usually appear on the trunk, but they can affect any part of the body. The condition is painless and there are normally no other symptoms or additional problems.

'Linear' scleroderma means that the skin is affected in the form of a line, usually along an arm or a leg. The skin appears shiny, discoloured or scarred, and often feels tight and uncomfortable. In children, this should be monitored carefully because the ongoing growth of the limbs can be affected'

En coup de sabre' literally means 'cut of a sword'. This form of scleroderma presents on the scalp and temple in children. If the affected area is confined to the scalp then the problem is mainly cosmetic, although the underlying bone may be affected'

Parry Romberg syndrome' also affects children and presents on the face, and occasionally the tongue. This requires careful monitoring as it may affect the growth of the facial bones.

Systemic sclerosis

In systemic sclerosis (SSc), the internal organs are affected as well as the skin. This can include the heart and the oesophagus as well as the blood vessels, kidneys, lungs and digestive system. There are two types of this condition:

With limited systemic sclerosis, you may have lived with Raynaud's for a long time. It often progresses gradually, and usually only affects the face, the hands, the arms below the elbows, the feet and the legs below the knees - although the lungs and digestive system may become involved over time.

Diffuse systemic sclerosis is more likely to affect the whole body, and in some cases there can be potentially serious complications involving the heart, lungs and kidneys. Common symptoms include fatigue, joint pain and stiffness.

Causes and Outlook

We don’t completely understand what causes scleroderma, but it’s thought to be an autoimmune disease. This means your immune system, which usually fights off infections, goes wrong and starts attacking the tissues in your body.
With scleroderma, this leads the body to create too much collagen. As a result, the skin and sometimes the blood vessels and other parts of the body stiffen up, in a process called fibrosis

Scleroderma affects different people in different ways, the outlook will depend on the type and severity of your condition. For many people symptoms can be managed effectively, although some individuals who are more severely affected may be more restricted by their condition. Your doctor is the best person to advise upon what your diagnosis may mean for you.

How is Scleroderma Diagnosed

One of the frustrating things about scleroderma is that getting a diagnosis is not always straightforward. Because it’s a rare disease, it’s possible your GP has never seen a case before. And many other conditions can have similar symptoms. If they suspect scleroderma, your GP should refer you promptly to a specialist clinic. Unfortunately, there’s not a test that can tell, on its own, whether you have scleroderma or not. Your specialist team must put together lots of different information to confirm that you have the condition. They also need to determine what type of scleroderma you have, and find out which parts of your body are being affected. They will check your heart, lungs, digestive system kidneys and muscles, as well as your skin.

Living with scleroderma

For many people living with systemic sclerosis, managing their condition involves a combination of drug treatments, medical management and lifestyle changes. There may be a number of people involved in your care, including your doctor and nurse specialist, and they will be able to help you to find out what works for you. Often a multidisciplinary approach is needed, therefore if you need to be seen by another department, for example by a dietitian, your doctor can arrange for a referral.

Please follow the links below for more information on living with scleroderma and the various ways to help to manage the impact of your condition on your daily life.