Scleroderma

What is scleroderma?

Scleroderma means hard skin (in Greek, sclero is the word for hard and derma means skin). But there’s a lot more to this condition than having the odd patch of rough skin. And while it’s thought to be fairly uncommon, with an estimated 19,000 people diagnosed with scleroderma in the UK (i), more people may be affected than we think, since those with mild symptoms may not even mention them to their doctors.

Other facts about scleroderma include:

• Around 2.5 million people are affected worldwide (i).

• Symptoms of scleroderma most often start between the ages of 25 and 55 (though it can affect anyone of any age, including on rare occasions children) (i).

• Around four times more women are affected by scleroderma than men (i).

• Symptoms and their severity can vary widely, with no two cases being the same (i).

• Some types of scleroderma are mild and can even improve without any treatment, though others can be severe and even life threatening (ii).

• There is no cure for scleroderma, but the symptoms can usually be controlled with different treatments.

Scleroderma Diagnosis

There are two main types of scleroderma, both of which cause hard patches of skin. Doctors are still debating whether or not one form of scleroderma can progress to the other. Some even believe that, while there are similarities between the two types of scleroderma, they may actually be completely separate diseases (iii). Both types, however, are thought to involve problems with the immune system.

Scleroderma Diagnosis

This type of scleroderma is the mildest type and mostly affects the skin, though it can also spread to the muscles and bones beneath the skin. Up to half of all cases of localised scleroderma happen in children, who are 90 per cent more likely to have this type of scleroderma than the more severe type (iii) (see below).

We still don’t really know what causes localised scleroderma. However it may be the result of a problem with the immune system that makes cells in the skin called fibroblasts produce too much collagen. Experts think the immune system is involved because localised scleroderma often develops in people who have autoimmune conditions – where your immune system attacks your own body’s cells by mistake (iv). Other conditions that have been linked with localised scleroderma include Lyme disease, measles, pregnancy and skin injuries. It can also develop in people who are having radiotherapy. Often, however, there is no obvious cause.

There are a few different types of localised scleroderma, which can affect the skin in different ways. In all types the symptoms usually start gradually, with some tending to improve within a few years without any treatment. The most common types include:

• Morphoea (or morphea)
  
  If you have this type of localised scleroderma you will usually have patches of hard, dry skin anywhere on your body. The patches tend to be oval in shape and can range from 2 - 20cm across. The patches can be hairless and have a shiny appearance, starting off as purple in colour and gradually turning to white. Older patches can turn darker, but there are usually no other symptoms. Morphoea is mainly found in adults, while a similar type called superficial morphoea – which causes mauve-coloured patches often in skin folds such as the groin or armpits – is most often found in middle-aged women.

• Linear scleroderma
  
  Most commonly found on children’s arms and legs, this type of localised scleroderma causes a long strip or line of thickened skin that can feel tight and uncomfortable. The strips can also look shiny and discoloured, and in more severe cases the tissues beneath the skin can also be affected, causing scarring under the skin. According to the NHS, with linear scleroderma the underlying bone and muscle can occasionally be affected too (ii).

• En coup de sabre
  
  This is a deep form of linear scleroderma that affects the scalp and temple (en coup de sabre is French for sabre – or sword – cut). The strip of skin is hairless, and the bone beneath it can be affected (typically by shrinking).

• Generalised morphoea
  
  In this rare type of localised scleroderma you may have more patches in different parts of the body than with morphoea (if you have morphoea you may have just one to three patches but four or more patches with generalised morphoea). It can also cause widespread skin thickening on your trunk.
  
  Other rare forms of localised scleroderma include pansclerotic disabling morphoea, which is found in children and can affect bone growth; Parry-Romberg syndrome, which causes skin patches along with other symptoms such as facial pain, migraine and epilepsy; and Atrophoderma of Pierini and Pasini, which causes a significant loss of tissue beneath the skin, leading to indentations in the skin.

Systemic sclerosis

This type of scleroderma is more serious than localised scleroderma because internal organs can be affected as well as the skin. According to the NHS, systemic sclerosis mostly affects women and usually develops between the ages of 30 and 50, with children rarely affected (ii). It’s also thought to be more common in North America and Australia than it is in Europe (v).

Systemic sclerosis is classed as an autoimmune condition, which means antibodies produced by your immune system to fight germs such as viruses and bacteria attack some of your normal body cells instead (see What causes autoimmunity? below). If you have systemic sclerosis, your immune system attacks and damages your connective tissues – the tissues that support, bind or separate other tissues or organs in your body – as well as your blood vessels. This explains why it’s also classed as a connective tissue disease.

As with localised scleroderma, we still don’t know what triggers it – though since it’s thought to be more common in people who have the condition in their family, it’s possible that it’s an inherited problem. Other things that have been found to trigger it include some viruses, certain medicines, recreational drugs and chemicals including some pesticides, organic solvents and even hair dyes.

Two main types of systemic sclerosis

• Limited cutaneous systemic sclerosis
  
  People who have lived with a condition called Raynaud’s phenomenon can often develop limited cutaneous systemic sclerosis (limited scleroderma for short). This type of systemic sclerosis is the milder of the two, and mostly affects the skin on the hands, lower arms, feet, lower legs and face. Eventually, however, it can cause problems with the lungs and digestive system.
  
  Limited scleroderma starts and progresses slowly, causing patches of hard or thickened skin as well as red spots and hard lumps under the skin. Thanks to the way it progresses so gradually, you may not pay much attention to the symptoms until internal complications such as heartburn and problems with swallowing (dysphagia) start to appear.

• Diffuse systemic sclerosis
  
  Also called diffuse scleroderma, this is the type of systemic sclerosis that is most likely to affect your internal organs such as your heart, lungs or kidneys. It can also cause skin thickening anywhere on the body, as well as more general symptoms such as weight loss, fatigue and joint pain, stiffness and swelling. Unlike limited scleroderma the symptoms usually start suddenly and worsen quickly during the first few years. But after that the symptoms can level out and may even improve gradually.
  
  If, however, you have diffuse scleroderma that affects your internal organs it can cause a number of potentially serious problems, including high blood pressure, shortness of breath and pulmonary hypertension (high blood pressure in the lungs).

What is autoimmunity?

Your body has systems in place that regulate your immune system and make sure everything within it is working normally. When something goes wrong with this system, however, antibodies – proteins in your blood – and some types of immune cells can start attacking your tissues, causing an autoimmune condition. This can happen for a variety of reasons:

Family history

Genetics are thought to play a part in autoimmunity but experts still don’t quite understand how. There seems to be a hereditary component in some people with certain autoimmune conditions (vi), but many people with family members who have an autoimmune condition aren’t affected by autoimmunity themselves. In other words, if you have a family member with an autoimmune condition, it doesn’t mean you’ll definitely develop one too. It may well depend on whether or not other factors come into play as well.

Environmental factors

The fact that some people with a genetic predisposition to autoimmunity never develop an autoimmune condition does suggest something else is needed to trigger it. One of these things may be environmental factors such as exposure to toxins and chemicals. Indeed, some air pollutants and organic solvents have been linked with the development of certain autoimmune conditions (vii). And since some toxic compounds are thought to affect your genes, it’s possible – though not yet proven – that the combination of a genetic predisposition and exposure to environmental pollutants could be at least one of the things that may lead to autoimmunity.

Exposure to infections

Our immune systems are designed to protect us from infections, but it’s also thought that infections can sometimes lead to autoimmunity (viii).

Scleroderma treatment

There is no cure for scleroderma, but there are treatments that can help relieve the symptoms and stop the condition getting any worse. Some treatments are also available that help treat the complications of scleroderma, particularly diffuse scleroderma complications such as high blood pressure and pulmonary hypertension.

The most common type of localised scleroderma, morphoea, often doesn’t need any treatment and can clear up on its own. But there are creams and ointments that can help stop it spreading, such as steroid creams for instance.

Medicines often used to treat other types of localised scleroderma include high-dose steroids, medicines designed to calm an overactive immune system, ultraviolet light therapy and physiotherapy in cases where the skin is very tight or where there’s scarring underneath the skin.

If you have systemic sclerosis your GP or specialist may prescribe medicines that help relieve your systems – moisturisers that help with dry or tight skin, for instance – and drugs that help stop the condition progressing too quickly. You may also be prescribed medicines that treat any complications that have been caused by systemic sclerosis, and you may need physiotherapy to help keep your muscles strong and your joints mobile.

There’s lots more information on scleroderma treatments on the Scleroderma & Raynaud’s UK website.

Tips on how to help yourself

While there is a wide range of medical treatments that can make living with scleroderma easier, you can also do things yourself to make life more comfortable. For instance, many people with scleroderma find they get tired easily, so if that happens to you try to take a break whenever you need to. Try to make sure you’re getting plenty of sleep – if you’re having problems getting the rest you need, take a look at the tips in our guide to insomnia.

The NHS also says it’s important to exercise and stop smoking (if you smoke) to keep your blood pressure under control and improve your circulation (ii). Eating healthily is also a good idea, whether you have gastro-intestinal symptoms or not (according to Scleroderma & Raynaud’s UK, up to 90 per cent of people with systemic sclerosis find that the condition affects their digestive system (ix)).

What is the best diet for Scleroderma?

The charity also says many people living with scleroderma experience symptoms that can lead to a poor appetite and weight loss, which makes choosing a healthy balanced diet essential. Some of the important nutrient the charity says you should make sure you’re getting plenty of include:
 

• Calcium (to keep your bones healthy, make sure you eat some milk and dairy products each day, or dark green vegetables, pulses, seeds, nuts and fortified cereals if you’re a vegan).

• Vitamin D (needed to help your body absorb and use calcium, vitamin D is mostly obtained from sunlight, though you can get small amounts in some foods such as eggs, butter and margarine spread and fortified foods – also see supplement information, below).

• Iron (animal sources of iron include red meat, poultry, fish and eggs, but you can get it in plants too, including dark green leafy vegetables).

• Omega-3 fatty acids (the most beneficial omega-3 fatty acids are found in oily fish such as sardines, salmon, fresh tuna, pilchards and mackerel as well as vegan omega-3 supplements (see below), though you can get other omega-3 fats in foods such as nuts and seeds too).

What vitamins are good for scleroderma?

If you’re living with scleroderma eating a healthy diet can help support your immune system. However few of us have the perfect diet all the time. If your diet isn’t as healthy as it should be, certain nutritional supplements that may be useful, including a quality multivitamin and mineral supplements with good levels of zinc, iron, vitamins A, D, E, K, B12 and folic acid.

Other nutritional supplements that may help give your immune system an extra bit of support include beta glucans, natural compounds found in foods including grains, baker’s yeast, seaweed and some mushrooms. Indeed, researchers have found beta glucans may help trigger and enhance the function of immune cells (x).

Some nutritional supplements may also be helpful for those with autoimmune conditions:

• High-strength fish oils: The omega-3 essential fatty acids found in oily fish such as salmon, fresh tuna, sardines, mackerel, herring and pilchards may be helpful in many autoimmune conditions, since it’s thought fish oils may help improve skin health as well as reduce joint and muscle pain (xi).
  
  If you don’t like eating fish you may like to try a high-strength fish oil supplement. If you’re a vegetarian or vegan you can still benefit from an omega-3 supplement, thanks to the availability of products that contain the natural triglyceride (TG) form of omega-3 This is sourced from plant organisms called microalgae rather than fish.

• Vitamin D: Try to make sure you’re getting plenty of vitamin D, since experts believe vitamin D deficiency may be associated with increased autoimmunity (xii).
  
  Vitamin D deficiency is thought to be common in some countries including the UK, which is why the Scientific Advisory Committee on Nutrition advises that adults and children over the age of four should consider taking a daily supplement containing 10mcg of vitamin D during the autumn and winter months, and indeed throughout the year if they avoid sun exposure or keep themselves covered up in the summer (xiii).
  
  The recommended form of vitamin D is vitamin D3 or cholecalciferol, as it’s the natural form of vitamin D that the body makes when it’s exposed to sunlight. Vitamin D3 supplements are available in tablet form, and now you can get them in veggie-friendly drops too.
  
  However, most vitamin D3 supplements are made from the fat of lamb’s wool, which means they’re unsuitable for vegans. The good news is that vegan vitamin D3 supplements sourced from lichen are now more widely available.

Living with scleroderma can be a challenge, especially if you have the systemic type. But as this guide shows there are things you can do to feel better and live life to the full. You can read more about conditions that affect your skin in our skin care pages, as well as find out more about immune system health in our immunity pages Also, for information about a wide range of health issues, visit our pharmacy health library .

References:

1. Available online: https://www.sruk.co.uk/scleroderma/what-scleroderma/

2. Available online: https://www.nhs.uk/conditions/scleroderma/

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4. Available online: https://www.sruk.co.uk/scleroderma/what-scleroderma/localised-scleroderma/

5. Available online: https://patient.info/skin-conditions/scleroderma-systemic-sclerosis

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9. Available online: https://www.sruk.co.uk/scleroderma/managing-scleroderma/nutrition/

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Disclaimer: The information presented by Nature's Best is for informational purposes only. It is based on scientific studies (human, animal, or in vitro), clinical experience, or traditional usage as cited in each article. The results reported may not necessarily occur in all individuals. Self-treatment is not recommended for life-threatening conditions that require medical treatment under a doctor's care. For many of the conditions discussed, treatment with prescription or over the counter medication is also available. Consult your doctor, practitioner, and/or pharmacist for any health problem and before using any supplements or before making any changes in prescribed medications.

 

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