In my senior presentation my topic is Scleroderma, as most of you know scleroderma is a chronic disease the affect the connective tissue and most of the organs of the body this disease is not captious, inherited cancerous or malignant. Scleroderma affect about 300.000 people that are affect with localized and systemic, These disease affect most the skin on the hands, feet, arms, and etc.Scleroderma is still unknown until this day about cure that can help patients with scleroderma. There are few medication help external problem that most patients have on an everyday basic. I talked how scleroderma can affect people life and how this disease can affect people personal life also. Scleroderma has to very cautious about this disease because it is for a life time. Scleroderma appearance more in women then man, more likely in infant with a small immune system.

Scleroderma

Introduction

Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases. The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of the disease. There are two types of scleroderma localized in which are the skin tissues that don’t harm any major organs it may get or go away without any specific help. Systemic types that have been known to affect the skin tissues under the blood vessels and other major organs. Which later in this comparatives studies localized and systemic will be discussed. The disease has been called “progressive systemic sclerosis,” but the use of that term has been discouraged since it has been found that scleroderma is not necessarily progressive. The disease may take several forms which will be explained later. There is also much variability among patients. Scleroderma is not contagious, it is not infectious, and it is not cancerous or malignant. Any chronic disease can be serious. The symptoms of scleroderma vary greatly from individual to individual, and the effects of scleroderma can range from very mild to life-threatening. The seriousness will depend on what parts of the body are affected and the extent to which they are affected. A mild case can become more serious if not properly treated. Prompt and proper diagnosis and treatment by qualified physicians may minimize the symptoms of scleroderma and lessen the chance for irreversible damage. The diagnostic process may require consultation with rheumatologists (arthritis specialists), and/or dermatologists (skin specialists) and require blood studies and numerous other specialized tests depending upon which organs are affected. There are an estimated 300,000 people in the United States who have scleroderma, about one third of whom have the systemic form of scleroderma. Since scleroderma presents with symptoms similar to other autoimmune diseases, diagnosis is difficult and there may be many misdiagnosed or undiagnosed cases as well. Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults. Overall female patients outnumber male patients about four to one, and the average age at diagnosis is in the forties. Factors other than sex, such as race and ethnic background, may influence the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. The reasons for this are not clear. Although scleroderma is not directly inherited, some scientists feel there is a slight predisposition to it in families with a history of rheumatic diseases. However, scleroderma can develop and is found in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55. When doctors say “usually” or “for the most part,” the reader should understand that variations frequently occur. Many patients get alarmed when they read medical information that seems to contradict their own experiences, and conclude that what has happened to them is not supposed to happen. There are many exceptions to the rules in scleroderma, perhaps more so than in other diseases. Each case is different, and information should be discussed with your own doctor. The exact cause or causes of scleroderma are still unknown, but scientists and medical investigators in a wide variety of fields are working hard to make those determinations. It is known that scleroderma involves overproduction of collagen. Most patients do not have any relatives with scleroderma and their children do not get scleroderma. Research indicates that there is a susceptibility gene which raises the likelihood of getting scleroderma, but by itself does not cause the disease. At the present time, there is no cure for scleroderma, but there are many treatments available. Some are directed at particular symptoms like heartburn, which can be controlled by medications called proton pump inhibitors or medicine to improve the motion of the bowel. Some treatments are directed at decreasing the activity of the immune system. Some people with mild disease may not need medication at all and occasionally people can go off treatment when their scleroderma is no longer active. Because there is so much variation from one person to another there is great variation in the treatments prescribed.

What are the different types of scleroderma?

The group of disease we call scleroderma falls into two main classes: localized scleroderma and Systemic sclerosis.

Localized Scleroderma

Localized types of scleroderma are those limited to skin and related tissues and in some case the muscle below. Internal organs are not affected by localized scleroderma and localized scleroderma can never progress to systemic form of the disease. Often localized conditions improve of go away on their own over time; but the skin damage that occur when the disease is active can be permanent. For some person is very serious and can disable if they are affect.

These are two generally recognized types of localized scleroderma:

Morphea: Morphea that comes from the Greek word that mean “form” or “structure”. The word refers to local patches of scleroderma. The first signs of the disease are reddish patches of skin thicken into firm, oval-shaped areas. The center of each patch become ivory colored with violet borders. Sometimes these appear on the face, arms, and legs. This disease is a simple plaque-type.

Linear scleroderma: As suggested by its name, the disease is characterized by single line or band of thickened or abnormally colored skin. Usually the line runs down an arm or leg, but in some it shown on their forehead.

The next is called Systemic Scleroderma better known as Systemic Sclerosis

Systemic Sclerosis: Systemic Sclerosis is typically broken down into limited coetaneous scleroderma and diffuse coetaneous. Some doctors break systemic down into three subset. This means that patients have other manifestation of scleroderma but they do not have any overt skin thickening.

Limited coetaneous scleroderma: this disease gradually and affect the skin only as: the finger, face, lower arms and legs. Most people with limited disease have Reynaud’s phenomenon for year before skin thickening start. This disease involves with the gastrointestinal that commonly and some patient have severe lung problem People with limited disease often have severe lung disease. Doctor calls this disease CREST:

Calcinosis: the formation of calcium deposits in the connective tissues, which can be detected by x ray. These deposits are typically found on the fingers, hands, face, and trunk and on the skin above elbows and knees. When the deposits break through the skin, painful ulcers can result.

· Raynaud’s phenomenon: a condition in which the small blood vessels of the hands or feet contract in response to cold or anxiety. As the vessels contract, the hands or feet turn white and cold, then blue. As blood flow returns, they become red. Fingertip tissues may suffer damage, leading to ulcers, scars, or gangrene.

· Esophageal dysfunction: impaired function of the esophagus (the tube connecting the throat and the stomach) that occurs when smooth muscles in the esophagus lose normal movement. In the upper and lower esophagus, the result can be swallowing difficulties. In the lower esophagus, the result can be chronic heartburn or inflammation.

Sclerodactyly: thick and tight skin on the fingers, resulting from deposits of excess collagen within skin layers. The condition makes it harder to bend or straighten the fingers. The skin may also appear shiny and darkened, with hair loss.

Telangiectasia: a condition caused by the swelling of tiny blood vessels, in which small red spots appear on the hands and face. Although not painful, these red spots can create cosmetic problems.

Diffuse coetaneous scleroderma: This condition typically comes on suddenly. Skin thickening begins in the hands and spreads quickly and over much of the body, affecting the hands, face, upper arms, upper legs, chest, and stomach in a symmetrical fashion (for example, if one arm or one side of the trunk is affected, the other is also affected). The damage of diffuse scleroderma typically occurs over a few years. After the first 3 to 5 years, people with diffuse disease often enter a stable phase lasting for varying lengths of time. During this phase, symptoms subside: joint pain eases, fatigue lessens, and appetite returns. Progressive skin thickening and organ damage decrease.

Materials and Method

The diagnosis of scleroderma is made by a medical history, physical examination and diagnostic tests that examine the blood and pulmonary function studies for the lungs, chest x-rays, studies of gastrointestinal function and electrocardiography EKG of the heart.

Depending on your particular symptoms, a diagnosis of scleroderma may be made by a general internist, a dermatologist (a doctor who specializes in treating diseases of the skin, hair, and nails), an orthopedist (a doctor who treats bone and joint disorders), a pulmonologist (a lung specialist), or a rheumatologist (a doctor specializing in treatment of musculoskeletal disorders and rheumatic diseases). A diagnosis of scleroderma is based largely on the medical history and findings from the physical exam. To make a diagnosis, your doctor will ask you a lot of questions about what has happened to you over time and about any symptoms you may be experiencing. Are you having a problem with heartburn or swallowing? Are you often tired or achy? Do your hands turn white in response to anxiety or cold temperatures(Mayo Clinic 2007b)

Once your doctor has taken a thorough medical history, he or she will perform a physical exam. Finding one or more of the following factors can help the doctor diagnose a certain form of scleroderma:

changed skin appearance and texture, including swollen fingers and hands and tight skin around the hands, face, mouth, or elsewhere

Calcium deposits developing under the skin

Changes in the tiny blood vessels (capillaries) at the base of the fingernails

Thickened skin patches.

Finally, your doctor may order lab tests to help confirm a suspected diagnosis. At least two proteins, called antibodies, are commonly found in the blood of people with scleroderma:

Antitopoisomerase-1 or Anti-Scl-70 antibodies appear in the blood of up to 30 percent of people with diffuse systemic sclerosis.

Anticentromere antibodies are found in the blood of as many as 50 percent of people with limited systemic sclerosis. Depending on your particular symptoms, a diagnosis of scleroderma may be

A number of other scleroderma-specific antibodies can occur in people with scleroderma, although less frequently. When present, however, they are helpful in clinical diagnosis and may give additional information as to the risks for specific organ problems.

Because not all people with scleroderma have these antibodies and because not all people with the antibodies have scleroderma, lab test results alone cannot confirm the diagnosis.

In some cases, your doctor may order a skin biopsy (the surgical removal of a small sample of skin for microscopic examination) to aid in or help confirm a diagnosis. However, skin biopsies also have their limitations: biopsy results cannot distinguish between localized and systemic disease, for example.

Diagnosing scleroderma is easiest when a person has typical symptoms and rapid skin thickening. In other cases, a diagnosis may take months, or even years, as the disease unfolds and reveals itself and as the doctor is able to rule out some other potential causes of the symptoms. In some cases, a diagnosis is never made, because the symptoms that prompted the visit to the doctor go away on their own.

Some patients have some symptoms related to scleroderma and may fit into one of the following groups:

Undifferentiated connective tissue disease (UCTD): This is a term for patients who have some signs and symptoms of various related diseases, but not enough symptoms of any one disease to make a definitive diagnosis. In other words, their condition hasn’t “differentiated” into a particular connective tissue disease. In time, UCTD can go in one of three directions: it can change into a systemic disease such as systemic sclerosis, systemic lupus erythematosus, or rheumatoid arthritis; it can remain undifferentiated; or it can improve spontaneously.

Overlap syndromes: This is a disease combination in which patients have symptoms and lab findings characteristic of two or more conditions.

Of three directions: it can change into a systemic disease such as systemic sclerosis, systemic lupus erythematosus, or rheumatoid arthritis; it can remain undifferentiated; or it can improve spontaneously (Adams 2003a)

Overlap syndromes: This is a disease combination in which patients have symptoms and lab findings characteristic of two or more conditions.

Observation and Result

What does Scleroderma look like?

Here are some picture of how scleroderma appear in the finger, stomach and lips

Symptoms

The following are some of the symptoms of scleroderma;

Ø Gradual hardening, thickening and tightening of the skin, usually in extremities such as ahands, face, and feet

Ø Skin discoloration

Ø Shiny skin

Ø Small white lumps under the surface of the skin that may erupt into a chalky white fluid

Ø Reynaud Phenomenon (pain numbness and or color change n the hand causes by spasm on the blood vessels upon exposure to cold or emotional stress

Ø Telangiectasia (real sports on the hands, palms forearms, face, and lips

Ø Pain and/or stiffness of the joint

Ø swelling of the hands and feet

Ø Itching may occur time to time

Ø Shortness of breath

Ø Weight loss

Ø Fatigue and weakness

Ø Digestive problems such as heartburn, difficult in swallowing diarrhea and conspitipation

Ø Hair loss

Treatment

Currently there is no cure for scleroderma and treatment involves altering specific symptoms associated with scleroderma:

Ø to treat skin thickening medication my include pencil amine

Ø To treat kidney problems medication to lower blood pressure such as vasodilators to lower angoifension-converting enzyme (ACE) in hibitors, and calcium channels blockers may help/

Ø T o treat gastrointestinal discomfort and infection, medication such as antacid H2 blocker (for example Tagment) omprazole and antibiotic may be help

Ø to treat esophageal disease and medication recommend by physician should be taken in liquid or crushed form.

Ø to treat joint pain and swelling, medication such as aspirin or non-steroid anti-inflammatory drugs (NSAIDS may help)

Research from the National Institute of Arthritis and Musoskeletal and Skin Disease (NIAMis), in which is a part of The United States Department of Health and Human Services’ National Institutes of Health (NIH).

Discussion

Causes

The causes of scleroderma is unknown

However we do understand a great deal about the biological process that involved. In localized scleroderma. The underlying problem is the production of collagen (scar tissue) in the involved areas of skin. In systemic sclerosis, there are three processes at work: blood vessels abnormalities, fibrosis, which are overproduction of collagen and the immune system dysfunction of collagen and the immune system dysfunction or autoimmunity. This is what responsible for Raynaud’s phenomenon and the painful; ulcers the can occur on the fingers. This vascular damage also occurs in the internal organs and is responsible for scleroderma renal crisis and ph.The small parties are normally capable or dilating (relaxing to adjust blood flow to the knees of the body. The thickening skin in scleroderma is caused by overproduction of the collagen which is his basic component of scar tissue. Abnormal articulation of collagen is called is ca; end fibrosis collagen is normal parts of skin and many organs. However scleroderma the balance of collagen formation and collagen formation and collagen break down is altered so that too much collagen build upon localized scleroderma this is confined to some area of the skin. Collagen is made by fibroblasts (a type of cell that is part of every tissue in the body. In which can be provoked or activated to make more. One way to detect activation of the immune system is to find antibodies which are protein made by the immune system cells, the bullets of our immune system in the blood target the body own tissue (antibodies. A very specific set of autoantibody is found in scleroderma. This autoantibody can be thought of as footprint of the scleroderma disease process under specific condition (Mayo Clinic Staff 2007b.)

Outbreaks

Ethnicity limited data in risk by ethnic groups in the United States suggest that the risk from highest to lowest is following:

Ø Choctaw Native American (highest)

Ø African American

Ø Hispanic

Ø Causcans

Ø Japanese American

African American has the highest reat of diffuse scleroderma, lung involvement and a worse prognosis that Causcans. Other studies also found lower survival rated among Japanese American

Genetics factors affect population groups differently. Studies are finding that ethnic group differs in the number of specific scleroderma. For instance Causcans have a higher rate of anti-cent mere antibodies, which are associated with limited disease, white African American patients have higher rate of anti-antibody and generic factor that are associated with more severe condition more in Native American.

Risk Factor

These are some main factors:

Ø Age. Systemic scleroderma usually develops between the age of 35 and 55. Localized scleroderma is more common in children then adults.

Ø Gender. The incidence of scleroderma is three to eight times higher in women than men

Ø Family history. A family history is the strongest risk factor for scleroderma, but even among family, the risk very low less than 1%

Genetics play a role that triggers the disease, but most causes are unlikely to be inherited Primary Research suggest patients with certain gene variation may be more susceptible to scleroderma those who do not calm these variations.

Prevention

There is no known prevention. Minimize exposure to silica dust and polyvinyl chloride.

There is several ways to keep scleroderma under control. Some patients or people who are affect with scleroderma have discomfort that comes with scleroderma is to take an active role in protection. Keep warm exposure to cold can trigger Raynaud’s phenomenon that cause the patient turn blue and your skin to show cracks. Develop a physical therapy program with your physician about to keep joints flexible. Wear protective joint equipment to avoid sprains and strains of joints. Always protect feet and hands for injury and infection. Moisturize the skin to keep it flexible. Some store brand has too much chemical and toxin that may cause the skin to crack or spread the scleroderma. Most of the time patients should get enough sleep least regular hours which is eight hours of sleep. Exercise to keep the heart in good help and to eat nutrition well balance diet (Mayo Clinic Staff 2003).

Conclusion

Scleroderma is a chronic disease that affects the connective tissue and hardening skin. There are about millions of people in the understand that are affect with this disease most of the patient have systemic. Scleroderma still doesn’t have any cure. In my presentation there are point where I discussion in this people. Patient who are affect show take very much cautious as I talk about in the Abstract, if patient is affect they seek physician or doctor for help.

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Literature Cited

Center of Disease Control and Prevention.2005a Feb3.Community-Associated MRSA

Information for the Public Department of Health and Human Service.

http://www.cdc.gov.

Mayo Clinic Staff 2007b Nov 9 MRSA Scleroderma and skin 1998-2008.Mayo Foundation for

Medical Education and Research (MFMER)

www.mayoclinic.com

Scleroderma Foundation

300 Rosewood Drive, Suite 105

Danvers, A 01923

Jacob Stanley, 1998. Signs and symptoms of Scleroderma .Medicine Net

http://www.medicinenet.com/sign/article .html