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- Digital ulcers
- Raynaud Phenomenon
- Swelling or puffiness of the hands
- Pain and stiffness of the joints
- Sclerodactyly and Joint Contractures
Decreased circulation to the fingers can result in skin breakdown at or near the tip of the finger resulting in painful ulcers. About 40% of SSc patients will develop at least one of these painful ulcers at some time in the course of this disease. These sores initially develop “spontaneously” due to lack of blood supply and are usually not due to an infection. However, if the area remains open for any length of time, it can become secondarily infected. Treatment of these ulcers include optimizing the circulation with medicine to dilate or open up the small blood vessels (vasodilators), being careful to keep the area clean and protected from injury. Typically, these ulcers can last for weeks and sometimes months.
Raynaud Phenomenon is the most common early symptom of systemic scleroderma. It is present at one time or another in about 90 percent of patients. It is most obvious in the fingers and toes but can also involve the ears, nose and tip of the tongue. In Raynaud Phenomenon, the blood vessels constrict or narrow in response to cold or emotional upset and stress. The resulting disturbance in blood circulation causes a series of color changes in the skin: white, blanched or pale, when circulation is reduced; blue as the affected part loses oxygen from decreased blood flow; and then red or flushed as blood flow returns and the part re-warms. Finally, as the attack subsides and the circulation returns to normal, usual skin color is restored. In the “white” or “blue” stages, sensations such as tingling, numbness and coldness may be felt. In the “red” stage, a feeling of warmth, burning or throbbing may be noted. Some people find Raynaud Phenomenon attacks painful.
Many common-sense preventive measures can be taken by those susceptible to Raynaud Phenomenon. Most obvious is minimizing exposure to cold, such as outdoor weather, air conditioning, or reaching into a refrigerator or freezer. Keeping your extremities and body warm is very important. Gloves or mittens should be worn, and a number of warming devices are available to protect the hands. Hats, ear muffs, heavy socks and warm, layered clothing of made from silk, cotton, wool and down feathers can help maintain body temperature. It is important to protect your hands with gloves when touching refrigerated or frozen items. Electric heaters, electric blankets and comforters can supplement the heat in the home or apartment. Keeping the entire body warm helps prevent Raynaud episodes. A warm bath or shower, or heating pad or hot water bottle on the back, may relieve an attack better than just warming the hands. Avoidance of emotional upset and stress can help but isn’t always possible. Many Various relaxation techniques, whether self-taught or learned through training courses, prove effective for some people to manage stress. One particular technique, biofeedback, has been used to increase finger temperature, but its benefit remains unclear.
Smoking definitely worsens Raynaud Phenomenon. For this and other reasons, persons with scleroderma should not smoke. Other aggravating factors may include concurrent carpal tunnel, estrogen, and stimulants. Your physician should review all your medications to ensure that you are not taking something that may make the Raynaud Phenomenon worse.
When Raynaud Phenomenon does occur, carefully waving the arms in an underhand, circular motion (like a softball pitcher) can help to restore blood circulation. Rubbing or massaging the hands and feet may also help.
To diagnosis Raynaud Phenomenon your physician may perform capillaroscopy by examining your nailfolds under a microscope to assess severity. Provocation testing by placing your hands in ice water to prove the presence of Raynaud’s should not be done. Physicians use a number of different medications to prevent, reduce the frequency or minimize the effects of Raynaud Phenomenon. Most of these drugs dilate or open up the blood vessels. Drugs commonly used include vasodilators such as prazosin, or calcium channel blockers such as amlodipine or nifedipine. Mild blood thinners such as aspirin, or drugs that decrease the stickiness of platelets, may improve circulation. There are now more than a dozen medications to improve circulation, and it is not possible to list them here. If you are receiving medication for pulmonary arterial hypertension, these medications may help in the treatment and prevention of Raynaud Phenomenon attacks.
Raynaud Phenomenon is not confined to people with scleroderma. It is also seen in people with lupus, rheumatoid arthritis and other connective tissue diseases. In addition, many healthy people have Raynaud Phenomenon without any other illness. In this situation, it is called Primary Raynaud Disease.
Calcium may deposit in the soft tissues of the hand and is different from a digital ulcer. These white, chalky deposits can drain from the surface of the skin and can commonly be inappropriately treated with antibiotics. While there are no good established treatments for calcinosis, surgical removal can be an option.
or puffiness of the hands
Swelling is another typical early symptom of scleroderma, and this may be especially noticeable upon waking up in the morning due to muscle inactivity at night. The skin of the fingers may look full and sausage-like, making it difficult to close the hand into a fist. Exercising the fingers and toes can help. Your physician may recommend medications to reduce inflammation. Referral to an occupational therapist at this stage may be helpful for range of motion education (see below).
and stiffness of the joints
Symptoms of pain, stiffness, swelling, warmth or tenderness may accompany the arthritis-like joint inflammation which frequently occurs in scleroderma. Muscle pain and weakness are other important symptoms. Aspirin or aspirin-like medications (non-steroidal, anti-inflammatory prescription drugs) can help. Hand radiographs or joint ultrasound may be performed by your physician in order to determine if joint inflammation requires additional therapies.
and joint contractures
Sclerodactyly means “hard skin of the digits,” particularly the fingers and toes. It generally occurs after initial swelling has subsided. It is characterized by shiny, tight skin of the fingers.
Affected digits may be difficult to move, and they may become fixed in a bent or flexed position called a “contracture” or a “flexion contracture.” Tightening and hardening of the skin and tissues surrounding the joints can cause decreased motion of the wrists, elbows and other joints.
“Range of motion” exercises performed daily are important to prevent or slow down the development of such contractures and to maintain limber joints. They may also help to increase blood supply to the tissues. These exercises are simple to perform and can be done at home. A typical exercise consists of laying the hand as flat as possible on a table, placing the heel of the other hand across the fingers, and gently pressing down to straighten the fingers. An occupational therapist can develop an exercise plan after consulting with your physician. He/she also may provide devices to help perform common personal care and household tasks more easily.