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Patient Education on Localized Scleroderma

 

What is Localized Scleroderma?

Localized scleroderma is a chronic disease of unknown cause with no known cure. Women are affected three to four times as often as men. Unlike systemic scleroderma, it is uncommon to find involvement of the internal organs. There are three types of cutaneous scleroderma: LOCALIZED MORPHEA, GENERALIZED MORPHEA, and LINEAR SCLERODERMA. Localized and generalized morphea usually appear between the ages of twenty to fifty, while linear scleroderma usually occurs at a much younger age. An important distinction between morphea and linear scleroderma is that linear scleroderma tends to involve not only the superficial, but also, the deeper layers of the skin and muscle, with fixation to underlying structures. The skin may be hardened. Localized scleroderma differs from diffuse scleroderma in the lack of Raynaud's phenomenon (bluish discoloration and pain of the digits), lack of acrosclerosis (thinning of the fingers), and internal involvement (difficulty swallowing).

 

What Causes Scleroderma?

The exact cause of scleroderma is unknown, but we do know that scleroderma is an immunologic condition in which the body mounts an autoimmune reaction to various components of human cells. In scleroderma, there may be a positive antinuclear antibody test, showing that the immune system is trying to attack the body's own cell nuclei. Other abnormal lab tests may include an abnormal anti single stranded DNA test, an abnormal rheumatoid factor, and abnormal anti histone antibodies. All of this may be very confusing to the non medical person, but in simple terms, scleroderma is an autoimmune disease due to a misguided immune system. White blood cells called T-cells are activated. Interleukins 2, 4, 6, and 8 are elevated. The result is excessive production and deposition of collagen in the tissues of the skin. In scleroderma, the fibroblasts (skin cells responsible for collagen production) are found to produce collagen at a faster rate than normal.

 

What are the symptoms?

Early morphea has an inflammatory stage, followed by one or more slowly enlarging patches or plaques. These plaques are most commonly oval in shape and vary in size. They have an ivory or yellow colored center and are surrounded by a violet colored halo. The halo signifies that the scleroderma is in a state of activity. Linear scleroderma shows sclerotic areas of skin in a linear distribution, often involving the muscle underlying the skin lesions. When linear scleroderma occurs on the face or scalp, the scarring resembles a saber wound, it is referred to as "en coup de sabre." Generalized morphea, a severe form of localized scleroderma, is characterized by widespread skin involvement with multiple sclerotic plaques, hyperpigmentation, and frequent muscle atrophy. The plaques may feel firm and hard, but are not deeply bound down. They may be depressed or slightly elevated and are seen more often on the trunk, but may also occur on the face and extremities. Generalized morphea may involve almost the entire skin surface. An uncommon form of morphea is the guttate variety. It is characterized by multiple, small chalk-white spots which vary in size from 1 to 10 mm in diameter. The violet colored line may surround all or some of the spots and in cases of long duration the line may be brown or grayish. Guttate morphea primarily involves the chest, neck and shoulders, and only occasionally other parts of the body. Localized morphea may last from a few months to many years. However, a large proportion of morphea patients improve spontaneously. Linear scleroderma is primarily a disease of childhood, and often causes more serious problems. Unlike morphea, linear scleroderma tends to be progressive. It generally begins in the first decade of life, and is occasionally associated with hemiatrophy (failure of development of one side of the body). Waxy appearing bands of sclerotic tissue develop which are usually limited to one area. Involvement of the extremities is common. It may extend ribbon-like from hip to toes or from shoulder to fingertips. There is often loss of deep tissue such as muscle and bone. It is most evident in children when the extremity fails to grow as rapidly as the uninvolved limb. Other symptoms that may occur are pain, swelling, stiffness of joints, positive antinuclear antibody test, and increased numbers of a particular type of white blood cell (eosinophils) in the circulation. While not every patient with morphea or linear scleroderma will require treatment, therapy may be indicated for patients whose lesions are so localized that further disfigurement or functional impairment may result from pressure atrophy secondary to the sclerotic involvement itself.

 

What Can Be Done?

Specific drug treatments are available, but the decision to treat may be difficult, especially in the case of children, as the side effects of treatment may be greater than one would like to accept. Numerous drugs have been used including: D-penicillamine, systemic corticosteroids, and antimalarials. In October, 1994, an article was published showing promising results with oral vitamin D (1,25 dihydroxyvitamin D3) Calcitriol in doses of 0.50 to 0.75 micrograms per day. Another therapy showing promise is the use of diphenylhydantoin (Dilantin) in relieving the atrophy and hide-bound appearance of linear scleroderma. Milder forms of morphea may be helped by cortisone injections of Celestone or Kenalog. Creams and emollients may help to soften the skin. Sunscreens are helpful in preventing lesions from darkening. Topical agents can make the skin cosmetically more acceptable. Therapy can also include physical and occupational therapy. Reconstructive surgery may be required to correct deformities. Systemic steroids are not routinely advised for mild cases of localized scleroderma, but occasionally, small doses of prednisone are beneficial. Systemic sclerosis rarely develops from localized scleroderma, however, it is wise for patients with generalized morphea to have a thorough examination. Dr. Jacobs may refer this type of patient to a rheumatologist for a second opinion. A rheumatologist specializes in autoimmune diseases.

 

Additional information on scleroderma.

There are several different forms of scleroderma. Localized scleroderma affects mainly the skin. It can affect skin in different areas of the body. It may also affect muscles and bone, but it does not affect internal organs. This form is usually not as severe as generalized scleroderma. People who develop localized scleroderma usually do not develop generalized scleroderma. There are two types of localized scleroderma: morphea and linear. Morphea happens when hard, oval-shape patches form on the skin. The patches are usually whitish with a purplish ring around them. They usually occur on the trunk, but can also occur on the face, arms, legs, and other parts of the body. Morphea often improves by itself, over time. Linear is a line of thickened skin that occurs in areas such as the arms, legs, or forehead. It can occur in more than one area. The line can extend deep into the skin and affect the bones and muscles underneath it. This can affect the motion of joints and muscles, as well as the growth of the affected area. When the line forms a long crease on the head or neck, it is sometimes called en coup de sabre - the strike of a sword. Linear scleroderma usually occurs in childhood. Generalized: Generalized scleroderma affects mans parts of the body. It can affect the skin as well as internal body parts, such as blood vessels, the digestive system (esophagus, stomach, and bowel), the heart, lungs, kidneys, muscles, and joints. The severity of this form depends on the organs affected and how much they are affected. In rare cases, scleroderma may affect only some internal system, leaving the skin and joints untouched. There are two types of generalized scleroderma: limited (also called the CREST syndrome) and diffuse. CREST stands for a combination of symptoms: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia. This type usually has a slow onset, with the first symptoms appearing 10 to 20 years before the full syndrome occurs. It usually affects the skin on the face, fingers and hands. Later on, it may affect internal organs, such as the esophagus (the tube leading from mouth to the stomach), the lungs and bowels. Diffuse occurs throughout the body. It usually affects the skin as well as other body parts, such as the lungs, kidneys, heart, bowels, blood vessels and joints. Depending on the areas affected, this type can cause problems such as high blood pressure, muscle weakness, trouble swallowing, or shortness of breath. Diffuse scleroderma may progress slowly in some people and more rapidly in others. However, with proper management, it can usually be controlled. The cause of scleroderma is unknown. It is not contagious, so you can't catch it from someone or give it to anyone. It is not inherited or passed on from one generation to the next, except in rare instances. We do know that in scleroderma the body produces too much of a protein called collagen. This excess collagen is deposited in the skin and in body organs. This causes thickening and hardening of the skin and affects the function of internal organs. Scientists think the body's immune system plays a part in causing excess collagen deposits. The immune system, your body's natural defense against disease, normally develops antibodies. Antibodies are protective substances in the blood that fight off invading organisms (like harmful bacteria and viruses). But sometimes the antibodies attack the body's healthy tissue instead. This abnormal process is called an autoimmune reaction. We know that the small blood vessels are damaged in scleroderma. There may be a connection between the build-up of excess collagen and blood vessel changes. Researchers are trying to find the answer to this as well as to the immune system's role in the disease. Scleroderma is a rare disease. It affects women two to three times more often than men. The disease usually starts between ages 30 and 50. It is sometimes seen in children and the elderly. Scleroderma affects everyone differently. It can be mild in some people, and more severe in others. Each form of scleroderma affects the body in different ways. Below, are some of the ways the disease may affect the body. Skin Changes: Skin changes occur in most people with scleroderma: * hardening and thickening of skin, especially on the hands, arms, and face * ulcers on the fingers * decrease in hair over the affected area * change in skin color. Swelling: Swelling or puffiness of the hands and feet is another common symptom of scleroderma. This often happens in the morning. You may notice: * skin on fingers and toes may look and feel swollen * shiny skin * usual skin creases disappear * tight skin - may be difficult to make a fist * numbness and tingling in fingers Sclerodactyly: Sclerodactyly means "scleroderma of the digits" (fingers and toes). It usually occurs after the initial swelling goes away. You may notice: * skin on fingers and toes becomes hard and shiny * difficulty bending fingers * contractures in fingers Raynaud's Phenomenon: Raynaud's phenomenon is a problem of poor blood flow to fingers and toes. Blood flow decreases because blood vessels in these areas become narrow for a short time, in response to cold or to emotional stress. Sometimes all it takes to trigger this reaction is walking into a cold room or reaching into the refrigerator. This is an early and common symptom of scleroderma. What you may notice: * fingers, toes, and sometimes the tips of the ears, nose, or tongue, are very sensitive to cold * fingers turn bluish or very pale * tingling, numbness or a cold sensation in the fingers When the hands warm up, the blood vessels open and the skin color returns to normal as the blood supply to the fingers improves. A note about smoking: If you smoke, stop. In addition to all the other health reasons for not smoking, smoking can trigger attacks of Raynaud's phenomenon. Contact the American Cancer Society, American Heart Association, American Lung Association, or Dr. Jacobs to find out about ways to stop smoking. Telangiectasia: Telangiectasia happens when tiny blood vessels near the surface of the skin show through the skin. What you may notice: * small reddish spots on fingers, palms, face, lips, and/or tongue The spots are not harmful and can be hidden with cosmetics. Calcinosis Calcinosis happens when small white calcium lumps form under the skin. This is due to scleroderma, and is NOT caused by too much calcium in your diet. You may notice: * hard white lumps under the skin on fingers or other areas of the body. The lumps may break through the skin and leak a chalky white liquid. Arthritis and Muscle Weakness: These may also be symptoms of scleroderma Arthritis happens when joints become painful and swollen. A contracture happens when the skin and tissues around a joint become tight and hard causing the joint to tighten into a bent position. This can happen in the hands and other parts of the body. You may notice: * pain, stiffness, swelling, warmth and tenderness in joints * general fatigue * weak muscles, often in shoulder or hip Sjogren's Syndrome Sjogren's Syndrome is a decrease in secretions from the tear ducts, salivary glands and other areas of the body, such as the vagina. This happens if scleroderma affects the glands that produce these fluids. You may notice: * dry eyes and mouth * trouble speaking or swallowing food * dryness in the vagina or other areas of the body * increase in dental cavities. Digestive Problems: The digestive system includes the mouth, esophagus, stomach, and bowels. Scleroderma can weaken the esophagus and the bowels. It can also cause a build-up of scar tissue in the esophagus, which narrows the tube. You may notice: * difficulty swallowing * heartburn * bloating * nausea or vomiting * weight loss * diarrhea or constipation. Heart and Lung Problems: Scleroderma may cause the heart to slow down and, in some cases, can lead to heart failure or other problems. When the lungs are affected, they cannot function as well. You may notice: * shortness of breath * a persistent cough * chest pain. Kidney Problems: Scleroderma can cause high blood pressure and kidney failure. If not treated, this can be a serious problem. You should be aware of the signs of kidney problems, listed here. You may notice: * headache * shortness of breath * visual disturbances * chest pain * mental confusion. If you notice any of these signs, call Dr. Jacobs right away so the problem can be treated. The first step in diagnosis is a thorough physical examination and history of past and present symptoms. Laboratory tests and other studies may also be needed to help determine if you have scleroderma and whether it is present in internal organs. These tests can also rule out other diseases, but no single test will prove the presence of scleroderma. Tests may include a biopsy, during which a small piece of skin is removed and examined under a microscope. A radiologist may check the esophagus to detect digestive problems. Tests to see whether the immune system is working normally are usually done on a small sample of blood. There are also tests which help detect early lung problems. Since scleroderma affects everyone differently, Dr. Jacobs may need to see you more than once. You may also need to see other doctors, such as an arthritis specialist or a gastroenterologist. After studying your symptoms, test results, and the overall pattern of your illness, Dr. Jacobs will suggest a treatment program to manage the disease. This program will include: * medication * exercises * joint protection techniques * skin protection techniques * stress management. Medication: Although there's not yet a cure for scleroderma, there are many drugs that help control it. Some work well for some people and not at all for others, so what someone else is taking may not be right for you. * Aspirin may be used in large doses to treat joint pain and swelling. * Nonsteroidal anti-inflammatory drugs (NSAIDs) work like aspirin. They may be used to treat joint pain and swelling. * Steroids are man-made forms of a naturally occurring hormone in the body. They are sometimes used to treat muscle problems. The side effects of these drugs usually outweigh any slight benefit they might produce. * Antacids may be used to treat heartburn and to protect the esophagus. You may also be given drugs to decrease stomach acid, protect the stomach, or improve intestinal motion. * Blood pressure medication may be used to treat high blood pressure. It is extremely important to take the medicine the doctor prescribes for you even if you are feeling fine. High blood pressure is known as the "silent" disease and has no symptoms. Taking the medicine faithfully is the only way to keep it under control. * Drugs that increase blood flow to your fingers and toes may be used to treat Raynaud's phenomenon. * Other drugs may be used to treat the skin, or to control the disease. Contact your local Arthritis Foundation chapter for information about specific medications. Never change your medications without first talking to Dr. Jacobs. Exercise: Regular exercise helps improve overall health and fitness. For people who have scleroderma, it also helps keep the skin and joints flexible, maintain better blood flow, and prevent contractures. General exercise, such as swimming, cycling, or walking keeps you fit and flexible. Special range of motions exercises help keep skin and specific joints flexible. These should be practiced twice daily. You can do range of motion exercises by yourself or with help from a therapist or family member. Examples of range of motion exercises include: * stretching your fingers on a flat table top to help keep them from becoming stiff or fixed in one position * slowly opening your mouth as wide as you can to keep the facial area flexible. A physical or occupational therapist, or other health professional, can help you with these and other exercises. Joint Protection "Joint protection" means protecting swollen and painful joints from stresses and strains that can make them hurt more. Lifting or carrying heavy objects, for example, can strain and hurt your joints. Joint protection includes learning to perform daily activities in ways that will help your joints rather than strain them. Physical and occupational therapists can show you new ways to do activities, such as opening doors and drawers, getting out of chairs, carrying packages, ironing clothes, and brushing teeth. Joint protection may also include resting individual joints in removable, lightweight splints to help control inflammation. Splints should be well padded to avoid pressure on any areas of the skin. There are many devices that reduce stress on painful joints, which you can purchase or make at home. The Arthritis Foundation manual, Guide to Independent Living for People with Arthritis, describes such aids and their sources. Skin Protection The goal of skin protection is to keep a good supply of blood flowing to your skin, and to protect skin from injury. Dressing warmly will help do this. Keeping your body warm helps open the blood vessels in your arms, hands, legs, and feet. Here are some tips for keeping warm: * Wear gloves, especially if you have Raynaud's phenomenon. * Always wear a hat in cold weather, since much body heat is lost from an uncovered head. * Cover your face and ears with a scarf. * During cool weather, wear thin cotton garments next to the skin. The cotton layer "moves" the sweat away from the skin, and keeps the sweat from cooling your body * Wear wool: it's warmer than synthetic cloth like orlon. * Wear many thin layers: they'll keep you warmer than one thick garment. * Wear loose-fitting boots and shoes that won't cut off the blood supply and will allow you to wear warm thermal socks or layers of socks. Other ways to protect your skin: * Use a cold water room humidifier to keep skin moist. * Avoid using strong detergents or other substances that irritate your skin. * Try soap, creams, and bath oils that are designed to prevent dry skin, until you find the ones that give you the best results. * Enlist help from family and friends. In the winter, for example, let someone else get the paper from outside or start the car on a cold day. The same goes for reaching into the freezer or doing tasks that require putting your hands into cold water. Stress Management: There are emotional and social problems that come with having a chronic disease. Even if you can usually manage the illness, there may be times when you feel overwhelmed by problems. Emotional stress plays a part in reducing blood flow. To help reduce the effects of stress you should: * Get enough sleep. You may need to take short naps during the day. * Try to avoid situations that make you tense. * Try to keep feelings of anxiety and fear from getting the best of you. * Express your fears and anger about what is happening to you. It is often helpful to confide in family, friends, Dr. Jacobs, or a counselor. Family members also benefit from such open talks. If they can understand your problems and your feelings, they can help you deal with them. * Ask Dr. Jacobs to refer you to a social worker, counselor, support group, or community mental health center. * Contact your local chapter of the Arthritis Foundation for information about resources in your community. * Look into biofeedback training. It may help control Raynaud's phenomenon. Biofeedback may also help reduce pain and promote relaxation. Talk to Dr. Jacobs about whether it would be helpful to you and where to get this training. Other Considerations Climate: A warmer climate will not cure scleroderma, but it may cut down on the occurrence of Raynaud's phenomenon and reduce the risk of developing ulcers on your fingers. If you consider moving to a warmer climate, you'll need to decide if the benefits outweigh the expenses and complications of relocating. Dental Care: Sjogren's Syndrome can cause an increase in cavities and tooth decay. Prevent tooth and gum problems by brushing and flossing your teeth daily and by visiting a dentist regularly. Diet: No diet will cure scleroderma, but you should eat balanced meals and stay at a sensible weight. If you have trouble swallowing, eat slowly and chew thoroughly. Also drink water or another beverage to soften food. Eat high fiber foods to help cut down on constipation. Try eating six small meals a day rather than three large ones, so food digests better. To help prevent stomach problems: * Avoid foods that cause heartburn or gas, such as spicy foods. * Use antacids for relief from heartburn. Do not lay down for about four hours after a large meal. * Eat your largest meal in the middle of the day rather than close to bedtime. * Raise the head of your bed by putting six-inch blocks under the end. This will help keep stomach acid from washing into your esophagus while you sleep. Scleroderma should not stop you from having a loving relationship or having sex. Yet, because of the illness, there may be some emotional and physical concerns to consider. Emotional Concerns: Scleroderma may change the way you feel about yourself You may feel you're not as attractive or fun as you used to be. You may feel less sure about your relationship with your partner. Yet, while scleroderma may change the way you look to some degree, it does not have to change the person you are. Also consider your partner's feelings. If your partner is concerned about the fatigue, pain, discomfort, and emotional stress the illness causes you, he or she may hesitate to have sex. Discuss these feelings with your partner. Through honest sharing, you can find ways to solve these problems. Although sex can play an important part in a relationship, it's love and caring that enrich the relationship. If sexual attraction decreases, it does not mean that love has decreased, also. Physical Concerns: There are many things you can do to handle physical problems that may arise. If fatigue is a problem either before or during sex, try making love at different times during the day. Also pace yourself while making love, to save energy. If joint pain is a problem, try using different positions while making love. A warm bath beforehand, and/or a waterbed or electric blanket may also be helpful. Some men may have problems with impotence, due to the illness. Talk to Dr. Jacobs about ways to treat this problem. Some birth control methods, such as a diaphragm or condom may be physically difficult to use. In such cases, your partner may be able to help you insert or apply the device. Also, some birth control pills may cause problems for women with poor blood flow. Talk to Dr. Jacobs before taking these pills, and ask about birth control methods that will work for you. We now know much more about treating scleroderma, and most people with this illness can continue to keep up with most of their normal activities. Most of what can be done to manage scleroderma depends on your own strong desire not to let it get the best of you, and on your common-sense approach to treatment. Think of yourself as being in a partnership with Dr. Jacobs. You both must work to keep the illness under control. Don't be afraid to ask questions. If there's something you don't understand, ask Dr. Jacobs to explain. The better you understand the disease, the more actively you can help yourself feel better.

 

Notes:

 
Ken Alpern, M.D. Charity Morris, PAC Eleni Litras,
PAC

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