Skin in systemic sclerosis

Raynaud’s is often the first noticeable sign of scleroderma, with 95% of people diagnosed reporting that Raynaud’s was their first symptom. It is the connective tissue disorder that is most associated with Raynaud’s.

Raynaud’s symptoms may include:

• cold fingers and toes
• colour changes in your skin, often from white, to blue, to red
• numbness
• tingling
• pain

Find out more on our Raynaud’s pages.

Tightening of the skin is a fundamental problem in systemic sclerosis. There are several medicines which may be of benefit for this problem, and these would normally be prescribed under the supervision of a hospital specialist. New medicines are constantly being developed.

In addition to drug treatments, physiotherapy and exercises are important to maintain skin movements around joints. Warm paraffin wax baths can also be useful to relieve tight, painful hands and to enable easier exercising. Soaking in warm water with a few drops of a liquid paraffin-based oil may help to soothe and relax painful hands and feet.

Many people, not just people with systemic sclerosis, suffer from dry and irritable skin. Dry skin is not healthy skin. Regular daily use of moisturisers can solve this problem. Moisturisers are either creams or ointments. Creams are based on water which means they are pleasant to use and soak into the skin well. However, they are not usually as effective as ointments which form a barrier on the skin and help to trap in your skin’s natural moisture.

Ointments are greasy and not as nice to use as creams. “Greasy” creams such as Diprobase® offer the best of both worlds and are to be recommended. Whilst there are many cosmetic moisturisers on the market, many containing extra additives, these are often very expensive and there is little evidence they offer any special benefit. Products containing more than 10% urea can be effective but can sometimes be irritating.

Bath emollients can be very helpful in keeping the skin moist, but beware as they can make the bath slippery. It is good practice to apply your chosen moisturiser once you have dried yourself after a bath.

Itchiness is another common problem for some people with systemic sclerosis. If regular moisturising as outlined above does not help, then other measures can be tried. Several creams are available with specific ingredients aimed to combat itch. These include Eurax cream®, E45 anti-itch cream®, Balneum Plus Cream® and Xepin Cream®.

In addition, antihistamine tablets may be tried. Some antihistamines can cause drowsiness and one needs to avoid driving etc. after they have been taken. However, it tends to be the antihistamines that cause mild sedation such as Atarax® which are the best at helping with itching, especially at night.

During the day, a non-sedating antihistamine such as Clarytin® could be tried. People often need to find which antihistamine suits them by trying several different ones. One further treatment which can be used is ultraviolet light therapy. This is only available in hospital dermatology units.

Self-treatment with home ultraviolet lamps is not advisable as the overall dose would not be monitored and one could cause lasting damage to the skin. An eight-week course of therapy can sometimes help with itching, although benefits may only be temporary.

Around 45% of people with systemic sclerosis develop ulcers. These are most common on the fingers and toes. Ulcers may also occur on the legs, often following trauma such as a knock with a shopping trolley. It goes without saying that one should take care to avoid such knocks, but accidents do happen. However, wearing trousers rather than a skirt, or a thicker pair of tights or socks may be enough to prevent the skin from being broken.

If a leg ulcer does develop it can take longer to heal in a patient with systemic sclerosis because the skin is not as healthy. Ulcers should be dressed regularly; the frequency with which the dressings are changed will vary from patient to patient. Sometimes an ulcer requires daily dressing but often two to three times per week is sufficient. Dressings help in several ways; they alleviate pain, contain any discharge, and prevent dirt and infection from entering the wound. It is also believed that there is an optimum moisture level to help with healing. An ulcer should be neither completely dry, nor too wet and sloughy. There are many different dressings available to try and provide the correct moisture level at the wound bed. Again, the choice of dressing will be dictated by the type of ulcer.

For ulcers on the fingers and toes a dry dressing is often all that is needed. Healing may be promoted by helping the blood to circulate freely using drugs such as vasodilators which can be given in oral forms such as Nifedipine, or intravenously such as Iloprost. The intravenous form is only available from certain specialist rheumatology units, but can have good effects on difficult ulcers on the fingers and toes. Ulcers may sometimes become infected and will require a course of oral antibiotics such as flucloxacillin. Signs that an ulcer has become infected are increasing pain, redness, discharge and smell. By eliminating infection one may be able to speed up healing. Ulcers can also develop eczema (inflamed skin) around them which may require a short course of steroid cream.

Calcinosis is often a debilitating and painful symptom of systemic sclerosis. It is the development of chalky, calcium material under the skin that leads to lumps and can result in pain or even ulceration. Although calcinosis is considered rare, it can affect between 20 – 40 % of those living with systemic sclerosis and is more common in patients with the limited form of scleroderma.

Sometimes the calcinosis breaks through the skin. It can occur at any site but is most often seen on the fingertips or at sites of pressure or trauma. Treatment is difficult and although there are some reports of medical therapies that have been helpful, such as the antibiotic minocycline that can help the body break down the calcified lumps, the most effective way of treating the problem is surgical removal.  However, calcinosis may recur, and it can be difficult to remove it completely. Also, when calcinosis developed close to nerves or blood vessels there can be risk of complications.

These are burst blood vessels visible as small red spots may develop on the face and hands. Understandably, many patients are keen for treatment. Pulsed dye laser treatment can often be helpful. This new technology utilises short bursts of high energy light to obliterate the broken blood vessels. This treatment will cause some stinging sensation on the skin and will leave some bruising for seven to ten days afterwards. It may take three to four treatments to work fully. This form of treatment is generally available from specialist hospital departments, and may require special funding.

Cosmetic camouflage is an effective alternative to cover the telangiectasia. Changing Faces offers tremendous help with this, and your local dermatology department can put you in touch with a trained volunteer.