What is Scleroderma?

Scleroderma means " hard skin " and is pronounced skler-o-derma.

Scleroderma is classified as a disease of the vascular and immune system, and a severe Scleroderma is classified as a disease of the vascular and immune system, and a severe ( hardening ) of the skin and multiple organs.

There are two major forms of Scleroderma.

1. Localized scleroderma

More common in children. Usually found in only a few places on the skin or in the muscles.

Rarely, if ever, does localized scleroderma develop into the systemic form of the disease.

There are two types Linear and Morphea.

Linear - Linear scleroderma is primarily a disease of childhood and often causes more serious problems. Unlike morphea, linear scleroderma tends to be progressive. It usually begins in the first decade of life, though it may occur with hemiatrophy ( failure of development of one side of the body ). The area of involvement may be extensive. Waxy appearing linear bands of sclerotic tissue develop. Involvement of the extremities is common: it may extend ribbon-like from hip to toes or shoulder to fingertips. Other symptoms that may occur include pain, swelling, stiffness of the joints, positive antinuclear antibody test, and increased numbers of a particular type of white blood cell ( eosinophiles ) Some of these patients later develop systemic rheumatic disease.

Morphea - Early morphea has a 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/yellow center and are surrounded by a violet colored area. The violet color signifies that the scleroderma is in a state of activity. The plaques 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 and can be confused with systemic scleroderma. 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. 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, some morphea patients improve spontaneously.

2. Systemic sclerosis

May affect the connective tissue in many parts of the body, e.g. the skin, the esophagus, gastrointestinal tract, lungs, kidneys, heart and other internal organs. It may also affect blood vessels, muscles and joints.

The types here are CREST, Limited and Diffuse.

CREST Syndrome ( limited Systemic Sclerosis)
C.....Calcinosis, refers to the formation of tiny deposits of calcium in the skin. This is seen as hard whitish areas in the superficial skin, commonly overlying the elbows, knees, or fingers. These firm deposits can be tender, can become infected.
R.....Raynaud's Phenomenon, is a condition that causes poor blood flow in one or more of the following, fingers, toes, ears, or tip of the nose. Sometimes, it may affect internal organs. Spasms of the small blood vessels cause changes varying from white to blue to red. Exposure to the cold or emotional stress can intensify the problem. There may be pain, tingling, numbness or a burning sensation.
E.....Esophageal Dysfunction, The loss of normal motility of the lower esophagus. This causes difficulty swallowing. A common complaint is acid reflux, with resulting heartburn. Should the esophagus become too restricted, dilation is the usually the treatment. To aid in overcoming reflux action, several small meals a day should be taken combined with small amounts of liquids. Do not eat before bed. Sitting upright one to two hours before bed is advised. Blocks may be used to raise the head of the bed six inches, and antacids may be prescribed for use an hour after meals and at bedtime.
S....Sclerodactyly, A condition in which the skin of the fingers becomes taut, thin and shiny. Fingers and toes may not bend or may become fixed in a flexed or less functional position. For this problem, physical or occupational therapy may be a valuable aid
T.... Telangiectasia, The manifestation of capillaries near the surface of the skin. They will blanch on pressure. ( turn white ) These capillaries become dilated and visible appearing mostly on the face. lips, tongue, fingers and palms,
Limited
This affects the hands mostly but could be in the face and neck. This is a milder form of scleroderma.
Diffuse
Is skin tightening above the wrist between the elbow and wrist. It may also just be internal affecting the connective tissue of the digestive system or some other internal organs such as lungs, kidney, heart and gastrointestinal tract
Causes of Scleroderma

The exact cause or causes of scleroderma are unknown.

It is known that the disease process in scleroderma involves an over-production of collagen.

People suffering from Scleroderma

Scleroderma affects more people than Multiple Sclerosis, Muscular Dystrophy or Cystic Fibrosis.

Approximately 4 times more woman then men develop the disease.

Although scleroderma strikes every age, sex and ethnic background, four times more females, during child bearing years, are afflicted with scleroderma than males.

Overlap

Patients can have variations of CREST, i.e. CRST, REST, ST, etc. Patients can also have "overlap" illness with features of both CREST and the diffuse form of scleroderma. Some patients have overlaps of scleroderma and other connective tissue diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and polymyositis. Approximately 10 percent of patients with the CREST variant develop elevated pressures in the blood vessels to the lungs (pulmonary hypertension).

Learning to Live With a Chronic Disease

 

Remember that you're not alone. In fact, you are a member of a large club. Almost half of all Americans have a chronic medical condition. Arthritis affects 42 million. High blood pressure is a problem for 22.5 million. Allergies affect 20 million. Some 16 million have diabetes. Heart disease afflicts 14 million. More than five million have asthma. The prevalence of chronic conditions means that help abounds: specialists, resources, books, counseling and support.
Take charge of your health. Think of yourself as the captain of your medical team. A chronic condition usually means contact with one or more medical specialists. But don't abandon your family doctor. That physician knows you best and can provide valuable perspective on what the specialists say. You might want to obtain e-mail addresses for all of your doctors and forward each doctor's recommendations to the others. The goal is for you and your doctors to come to a consensus on what treatment is best for you.
Learn about your condition. Understanding as much as you can about your condition helps alleviate anxiety. It also puts you in touch with valuable resources: specialists who can provide medical opinions, facts about treatment breakthroughs, products that can make your life easier. Fortunately, you have many resources at your fingertips.  Your Internet search engine can find many other sources for you, but sifting through hundreds of Web sites for information can be daunting. A research librarian can help you find reliable information, both on library shelves and on the Internet.
Join an organization. It's a very rare condition that doesn't have at least one national organization that educates the public. To find it, ask your physician, or request a referral to a medical social worker who can point you in the right direction. Another option is to go back to that librarian for help, or try an Internet search engine. 
 
Find a support group. Most organizations that focus on chronic medical conditions also sponsor support groups. These groups can provide emotional comfort, background information on your disease, opinions about treatments, recommendations to specialists, and friendship at a time when other friends and family members might not be able to meet your needs. In a support group, you get help, but you also give it, which gives you the feeling that you've regained control over your life. Several studies have shown that support groups actually extend the lives of people with cancer. If your condition limits your mobility, you can still participate in a support group by joining one online. Many of the condition-specific organizations sponsor online support groups through their Web sites. No matter what kind of chronic condition you have, join a support group, or launch one. The Self-Help Clearinghouse can help.
Eat a healthy diet. The National Cancer Institute urges all Americans to eat at least five servings of fruits and vegetables a day. Fruits, vegetables, beans, and whole grains are great dietary sources of antioxidant nutrients, notably vitamins A, C, and E. Antioxidants help prevent the cell damage at the root of many chronic degenerative diseases, such as heart disease, stroke and cancer. A diet high in antioxidants keeps your immune system working at peak efficiency, and the better your immune system, the healthier you become—despite your chronic condition. 
Try to get regular, moderate exercise. Exercise improves cardiovascular health, strength, stamina and flexibility. It helps control weight. It improves the quality of sleep. And it's a mood elevator, a natural antidepressant. For most people, the easiest exercise program to adopt is walking. Within the limits of your condition, try to be physically active for at least 30 minutes a day. Most people can manage a 30-minute walk around their neighborhood or a local park or mall. If you can't, consult your physician about the type of exercise you can do.
Watch out for substance abuse. No one should smoke, drink excessively (more than one or two drinks a day) or become dependent on drugs. This is doubly true for those with chronic conditions.
Get restful sleep. Ideally, a person needs seven to nine hours of sleep a night. If you have difficulty sleeping—and many chronic conditions interfere with sleep—consult your physician.
Reduce stress in your life. Stress and anxiety tend to exacerbate many symptoms, particularly pain. There are many paths to stress management: exercise, meditation, biofeedback, yoga, tai chi, prayer, and social support from family, friends or a support group. It also helps to do volunteer work, particularly with people less fortunate than you are. Pick the approaches that appeal to you. Ask your physician, social worker, family, friends or community organizations for referrals.
Consider complementary therapies. Depending on your condition, some complementary therapies might help you. Massage therapy, relaxation therapy, acupuncture are just a few possibilities. If you consult complementary practitioners, make sure you tell them what mainstream medical treatments you are using. You also need to tell your physicians what complementary therapies you're using. You get the best medical care with the fewest side effects when all of your practitioners know everything you're doing.
Rethink your limitations. There may be foods you can no longer eat, activities you can no longer participate in, and other changes in your life. It's perfectly normal to mourn the loss of things you can't do anymore. But if you stop there, you're bound to wind up feeling chronically blue. Look for new opportunities. Maybe you can't play three sets of tennis anymore, but perhaps you can sign up for that painting class you've never quite been able to fit into your schedule.
Remain hopeful. This may not be easy, especially shortly after your diagnosis. But research continues. Don't expect a miracle, but it's quite possible that in the not-too-distant future, a treatment advance might make your life considerably easier.

 

           Disclaimer ---  This site is for information only, for more information please seek the advice of  your doctor.