Raynaud's phenomenon is one of the earliest symptoms of scleroderma but occurs in other diseases as well. In Raynaud’s, the cells inside the blood vessel wall overgrow and narrow the opening of the blood vessel. This means that when people with Raynaud’s are exposed to the cold, change of temperature or undergo emotional stress, their blood vessels constrict, (become smaller and narrow) just like everyone else's, but since their blood flow is already decreased, the blood supply to their fingertips can be almost completely shut off. First the fingers look white (spasm). then they become blue (when there is a decrease in oxygen) and finally red (when the blood flow is restored).
It is unusual to develop digital ulcers in Primary Raynaud's but in secondary Raynaud's, (associated with scleroderma), repeated episodes of spasm of the fingers can cause pitted fingertip scars, and in some people this results in fingertip ulcers.
Until the day arrives when a medication is finally available that will prevent the blood vessels from clogging and also unclog damaged vessels in people with scleroderma, therapy has to be aimed at keeping the blood flowing into the fingers and toes and prevent infection of damaged skin and other tissues.
The best way to treat digital ulcers is to prevent them from occurring in the first place. This is not easy but there ways in which you may be able to minimise the risk:
Always be cautious about exposure to the cold. Make sure that you have gloves or mittens close to hand. It is also essential to keep the trunk of your body warm through wearing several thin layers of clothing. Heating aids can also prove a very important method of maintaining the circulation to the extremities or rewarming cold colds and feet.
Avoid pressure to sides and tips of fingers and do not be too aggressive when manicuring your cuticles.
Place hands alternately into warm and cool water as this helps to stimulate the circulation. However, be careful that the water is not too hot or too cold.
Do not carry heavy shopping bags as this can cause the fingers to go into a spasm due to the pressure of weight. Carrier bag handles may help but the same problem can occur if the bags are heavy. If you have to carry goods, a shoulder bag is a good alternative or use a shopping trolley.
Stop smoking! Nicotine causes the blood vessels to narrow. It has been proved that smoking one cigarette has been shown to decrease the temperature of the extremities by one degree over a twenty minute period. Passive smoking can also be dangerous so encourage anyone who lives with you to stop smoking and avoid places where cigarette smoke is likely to be present. Patients with scleroderma who smoke are three to four times more likely than are non smokers to incur digital vascular complications.
CAN MEDICATION HELP?
Medicines are available that can decrease ability of vessels to constrict with cold and emotional stress and other open up the blood vessels allowing the blood to flow more easily. For some people these medications will reduce Raynaud's attacks but in others it causes unwanted side effects as they open up the blood vessels throughout the body and not just in the extremities. This means that they can cause headaches, stomach problems and swelling of the ankles.
Some ulcers occur in scleroderma patients for no apparent reason, whereas in people with calcinosis they are caused by calcium deposits breaking through the surface of the skin. These ulcers can be extremely painful and therefore medications to relieve pain are essential to help people cope but should be on an intermittent basis. Always follow your GPs advice when using any medication.
If you develop an ulcer be very careful if it becomes infected. Any infection should be treated with antibiotics. Flucloxacillin, 500 mg 4 times a day for at least two weeks, is the medication of choice but if you are allergic to penicillin then there are alternatives. If there is any doubt regarding the infection, a swab can be sent to the laboratory for suitability of medication. Whereas previously patients were advised to soak their ulcers, it is not currently recommended or that any dressing is used except a clean dry dressing.
Wound healing is achieved by improving the supply of oxygen rich blood to the affected area. There are many creams and gels which are recommended including hydrogel dressings. These should be discussed with a wound care nurse if one is available.
The nurse may bring the ulcer to the attention of a doctor and instigate a review of your current medication. It may be possible that doses of vasodilators can be increased, or new vasodilators added. If ulcers are present for some months and are resistant to all attempts at healing then the patient may require a hospital admission for intravenous treatment of antibiotics or Iloprost, which is usually the first choice. Iloprost is currently used to control Raynaud's and is useful in the treatment of fingertip ulcers. Iloprost should be managed with care and the traditional dosage needs to be tailored to the phase of the disease in order to avoid side effects that may lead to discontinuation of the drug.
However, if this cannot be tolerated due to side effects similar to those of vasodilators, calcitonin gene related peptide (CGRP) may be used as an alternative.
A new oral treatment for digital ulcers which showed promise in a recent trial is by using bosentan. In the study, patients on bosentan developed significantly fewer ulcers than those on placebo (a dummy drug used for comparison) and also showed improvement in hand-related functions such as dressing, grooming, hygiene and grip. Sildenafil is another new option in the treatment of digital ulcers in scleroderma. As with bosentan, the major advantage compared with prostaglandin analogues is that it is taken orally.
If all non operative treatments have failed, a digital sympathectomy can be an effective procedure for treating non healing digital ulcers in scleroderma patients.
Use loose dressings on ulcers - finger bandages with an applicator are ideal for protecting the ulcers. It nay be advisable to wear a splint at times and worth discussing with your occupational therapist, especially if your fingers are curling. Ulcers can still occur on bent fingers, usually on the knuckles which is a difficult place to apply a dressing.
All ulcers take a long time to heal and therefore great patience is required. The are numerous creams, ointments, solutions and other medications that have been reported to heal fingertip ulcers. However, nothing works rapidly or consistently enough to convince all patients and doctors that the best single treatment has been discovered. The cure for fingertip ulcers will be that which can restore normal blood supply to the fingers. In the meantime, with good common sense and patience, these ulcers generally heal eventually for most people.
If you haven’t experienced these ulcers you can have no idea of the pain involved. Some ulcers occur in scleroderma patients for no apparent reasons whereas in people with calcinosis they are caused by calcium deposits breaking through the surface of the skin. You can bet that if you knock your hand it will be the finger with the ulcer on which gets the hit! the ulcers are always more painful when the hands are cold as it seems to get to the root of the ulcer and magnifies the pain. Best advice is early intervention as soon as an ulcer starts and to hang in there. They will heal eventually but it may take months or even years!
To download the Digital Ulcers & Skin Care leaflet click here.