Systemic Sclerosis Scleroderma Symptoms and Signs

Systemic Sclerosis Scleroderma Symptoms and Signs

Mentions getting an autopsy. I had stated they wouldn't find out what is wrong with me without an autopsy.

Scleroderma: eMedicine Rheumatology

Scleroderma: eMedicine Rheumatology

This link contains a VERY GOOD explanation of the symptom breakdown.

Systemic sclerosis. DermNet NZ

Systemic sclerosis. DermNet NZ


This link is very good. Here is the part I needed most.

Other skin changes include:

Itchy skin
Thickening of the skin of the fingers, then atrophy (thinned) and sclerosis (scarring). The fingers become spindle-shaped (sclerodactyly) from resorption of the fingertips.
Fragile nails become smaller with ragged cuticles
Taut, shiny skin that may have dark or pale patches (hyper- or hypopigmentation). The tight skin may affect most parts of the body, including the face, resulting in loss of expression and difficulty opening the mouth properly.
Visibly dilated blood vessels (telangiectases) appear on the fingers, palms, face, lips, tongue and chest.
Calcinosis (calcium deposits) develops in the skin, particularly the fingers, hands and other bony areas. These can breakdown and discharge chalky material.
Ulcers may follow minor injuries over the joints, or on the tips of fingers and toes where the circulation is poor. Ulceration can lead to dry gangrene and eventual loss of the tips of the fingers (like frost bite).
Ulcers may also arise over calcinosis and on the lower legs.
Sicca symptoms (dry eyes, dry mouth) and Sjogren syndrome
In addition to the skin changes, the disease affects many other organs. Problems that may occur include:

Friction rubs over the joints and tendons, particularly the knees.
Eye changes with tightness of lids, reduced tear secretion, retinopathy
Joint pain, muscle pain and weakness and limited movement resulting in contractures.
The digestive tract may be affected throughout its length. Oesophageal reflux is common causing difficulty in swallowing solid and liquid food. This can lead to nausea, vomiting, weight loss, stomach cramps, diarrhoea, constipation and bleeding.
Lung and heart involvement may manifest as shortness of breath, high blood pressure, chest pain, pleurisy, pneumothorax, pericarditis arrhythmias, general heart enlargement and heart failure.
Progressive kidney disease resulting in proteinuria, high blood pressure and eventually renal failure.

Scleroderma - Symptoms, Treatment and Prevention

Scleroderma - Symptoms, Treatment and Prevention


Definition of Scleroderma
Description of Scleroderma
Causes and Risk Factors of Scleroderma
Symptoms of Scleroderma
Diagnosis of Scleroderma
Treatment of Scleroderma
Questions To Ask Your Doctor About Scleroderma
Definition of Scleroderma
Scleroderma is a chronic, degenerative, autoimmune disorder that leads to the over-production of collagen in the body's connective tissue. The word "scleroderma" means "hardening of the skin" and refers to one of the possible physical effects of the disease.
If systemic (throughout the body), scleroderma is known as progressive systemic sclerosis.
Description of Scleroderma
Approximately 300,000 people in the U.S. have scleroderma. Women are affected three times as often as men. Although the disease can develop at any age, it most frequently appears in the third to fifth decades of life. It is most frequently a chronic and often progressive illness.
Collagen, a protein manufactured by the connective tissues of the body, is produced in excess in someone suffering with scleroderma. This over-production of collagen can be likened to the process of "scarring," which is the way the body heals a wound.
For persons with scleroderma, the production of collagen is abnormal, depositing it in various organs and/or tissues of the body, especially in layers of the skin.
It is important to stress that the symptoms of scleroderma vary greatly from person to person, as though each person with scleroderma has his/her own version of the disease. Although scleroderma can indeed be serious, most people are able to live a normal life span with varying degrees of discomfort and/or disability.
There are two forms of scleroderma: localized and generalized (also called systemic sclerosis).
Localized Scleroderma
affects mainly the skin in different areas of the body
may affect muscles and bone
does not affect internal organs
is not usually as severe as generalized scleroderma
does not usually develop into generalized scleroderma
Generalized Scleroderma
Generalized scleroderma affects the skin and/or internal body parts, such as blood vessels, the digestive system (esophagus, stomach, and bowel), the heart, lungs, kidneys, muscles, and joints.
Generalized scleroderma most often presents as either CREST syndrome or diffuse scleroderma.
CREST is an acronym derived from the syndrome's five most prominent symptoms:
C - calcinosis, painful calcium deposits in the skin
R - Raynaud's phenomenon, abnormal blood flow in response to cold or stress, often in the fingers
E - esophageal dysfunction, difficulty swallowing caused by internal scarring
S - sclerodactyly, tightening of the skin on the fingers and toes
T - telangiectasia, red spots on the hands, palms, forearms, face and lips
Diffuse scleroderma appears as thickening of the skin that often spreads from the fingers and hands to the face, trunk or major organs.
Other less common forms of scleroderma include chemically induced localized scleroderma, eosinophilic myalgia syndrome (a disorder caused by the ingestion of L-tryptophan), toxic oil syndrome (associated with contaminated oil), and graft-versus-host disease.
Effects Of Scleroderma On The Body
Scleroderma in the skin may cause the cessation of hair growth and sweating; and the tightening and thinning of skin over the knuckles or finger joints, resulting in ulcers (open sores) and curving of the digits.
Scleroderma in the kidney may cause severe hypertension (high blood pressure) and kidney failure.
Scleroderma in the gastrointestinal tract hampers the action of the smooth muscle that lines the esophagus, small intestine, and colon; resulting in swallowing difficulties, malabsorption of digested food into the circulation, severe constipation and/or possible intestinal obstruction.
Scleroderma in the lung makes the lungs less flexible and reduces their blood supply resulting in shortness of breath, susceptibility to bronchial problems, and pulmonary fibrosis (scarring and thickening of lung tissue).
Scleroderma in the heart and pericardium (fibrous sac that lines the heart) restricts the normal pulsation and pumping of the blood by encasing the heart in a shell of sclerosed tissue. This may lead to heart failure.
Scleroderma in the joints causes arthritic symptoms such as inflammation, pain, swelling, stiffness, redness, and, in some cases, joint deformity.

Causes and Risk Factors of Scleroderma
The cause of scleroderma is unknown. It is not contagious. It is not passed on from one generation to the next, except in rare circumstances.
Symptoms of Scleroderma
The following are some of the symptoms of scleroderma:
Gradual hardening, thickening, and tightening of the skin, usually in extremities such as hands, face, and feet
Skin discoloration
Numbness of extremities
Shiny skin
Small white lumps under the surface of the skin that erupt into a chalky white fluid
Raynaud's phenomenon (pain, numbness, and/or color changes in the hands caused by spasm of the blood vessels upon exposure to cold or emotional stress)
Telangiectasia (red spots on the hands, palms, forearms, face, and lips)
Pain and/or stiffness of the joints
Swelling of the hands and feet
Itching of the skin
Stiffening and curling of the fingers
Ulcers (sores) on the outside of certain joints, often the knuckles and elbows
Digestive problems such as heartburn, difficulty in swallowing, diarrhea, and constipation
Fatigue and weakness
Shortness of breath
Arthritis
Weight loss
Hair loss
Internal organ problems
Diagnosis of Scleroderma
The diagnosis of scleroderma is made by a medical history, physical examination and diagnostic tests.
The diagnostic tests may include skin biopsy (removal of a sample of skin for examination) and blood tests.
Other tests, such as pulmonary function studies for the lungs, chest X-rays, studies of gastrointestinal function, and electrocardiography (EKG of the heart) may be performed to determine the disease's severity and effect on the internal organs.
Treatment of Scleroderma
Currently, there is no cure for scleroderma and treatment involves alleviating specific symptoms associated with scleroderma.
To treat skin thickening, medications may include penicillamine.
To treat kidney problems, medications to lower blood pressure such as vasodilators, angiotensin-converting enzyme (ACE) inhibitors, and calcium channel blockers may help.
To treat gastrointestinal discomfort and infection, medications such as antacids, H2 blockers (e.g., Tagamet), omeprazole, and antibiotics may help.
To treat esophageal disease, any medication recommended by a physician should be taken in liquid or crushed form. Patients with esophageal reflux should avoid late-night meals and elevate the head of their bed 4 to 6 inches.
To treat joint pain and swelling, medications such as aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs) may help.
To treat joint and muscle stiffness, steroid medications, as well as heat therapy, physical therapy, and exercise may help.
To treat Raynaud's phenomenon, calcium channel blockers may help.
Protection
One way to reduce the discomfort that comes with scleroderma is to take an active role in protection.
Keep warm - exposure to cold can trigger Raynaud's phenomenon
Develop a physical therapy program with your physician to keep joints flexible
Wear protective joint equipment to avoid sprains and strains of joints
Protect feet and hands from injury and infection
Moisturize the skin to keep it flexible
Avoid using strong detergents, cleansers, and solvents
Get enough sleep
Reduce stress
Do not smoke
Questions To Ask Your Doctor About Scleroderma
Do any tests need to be done for diagnosis?

What type of scleroderma is it?

What areas or organs have been affected?

How serious is the condition?

What treatment do you recommend?

How successful is the treatment?

Can the disease progression be stopped?

What is the prognosis of this disease?

Is there a support group for scleroderma?

Living with Scleroderma

Living with Scleroderma


by Charlene Collins, Staff Writer (Ranked #2 expert in Immune System Diseases)

Scleroderma is a disorder caused by inappropriate responses from the immune system. The disorder causes thickening of the skin and connective tissue of the body. Scleroderma can also affect the blood vessels, lungs and other internal organs. The thickening of the tissues occurs because the body manufactures too much collagen. It’s not known why the body’s immune system attacks the skin and connective tissue. Scleroderma can occur in families, though, to date, no gene has been identified as the causative factor.

There are two different forms of scleroderma, which are localized and diffuse scleroderma. Localized scleroderma occurs in certain parts of the body; diffuse scleroderma is systemic. In other words, the diffuse form of scleroderma can affect the organs of the body, including the kidneys, lungs, bowels and esophagus.

Scleroderma of the skin and connective tissue can make movement very difficult. The skin and connective tissue becomes stiff and hard from the excess collagen being produced by the body. The overall health of an individual can suffer when her/his esophagus, lungs or kidneys become impaired. If the esophagus becomes stiffened, swallowing may become difficult. If the lungs are affected, the individual may suffer from respiratory insufficiency. When the kidneys are affected by scleroderma, filtration problems may occur. An individual may also have blood pressure problems if the kidneys and/or blood vessels are affected.

With scleroderma, an individual is likely to experience different complications associated with the disorder. The complications that could arise are related to hardening of the skin and circulatory problems.

Calcinosis

The medical term for tissue hardening is called calcinosis. Calcinosis is more likely to occur in the bend of the knees, fingers, elbows, toes, and other joints of the body. Calcinosis of the tissues can be painful, especially if the calcified pieces of skin break off. The areas which are calcified can become infected.

Raynaud’s phenomenon

Aside from hardening of the skin, an individual with scleroderma may be very sensitive to heat and cold. This sensitivity is called Raynaud’s phenomenon. Raynaud’s phenomenon occurs when the tiny blood vessels go through periods of spasm and relaxation. The areas on the body most often affected by Raynaud’s phenomenon are the fingers, toes, ears, nose, and tongue. These areas may experience burning like pain due to ischemia (lack of oxygen rich blood flow to the tissues).

Sclerodactyly

Like calcinosis, sclerodactyly occurs when the skin thickens and hardens so much that movement is difficult. The skin becomes shiny with calcified particles on the skin.

Conclusion

Individuals with scleroderma may have reddened areas on the hands, ears, lips and face. These reddened areas are caused by dilated capillaries in the affected areas. People who have diffuse scleroderma are more likely to have other serious medical conditions which are secondary to the disorder. For instance, acid reflux is quite common in people who have scleroderma in their esophagus. The stomach acid can splash up from the stomach into the esophagus, if the bottom third of the esophagus is affected.

People can lead relatively healthy lives if their internal organs are not affected. When people die from scleroderma it is usually due to the secondary conditions caused by the systemic form of the disease. However, most people live relatively normal life spans. Eating can become difficult if the jaw becomes stiff and difficult to move. If the bowel becomes stiff, an individual may be prone to having fecal impactions.

The disease is chronic and usually gets worse as time goes on. The degree of disability will vary from person to person; it will depend on the type of scleroderma and which parts of the body are affected.

Family support is important for people who are diagnosed with scleroderma. Living day to day with this disease can be challenging. Your skin can be painful and itchy all at the same time. It is easy to get depressed when your body slowly becomes immobile. One of my aunts had scleroderma. She lived a long life; she had good days and bad days, but she got progressively worse until she died. The main thing I remember about her was that her hands looked hard and shiny.

Sources:

http://www.medicinenet.com/scleroderma/article.htm

http://www.healthscout.com/ency/68/311/main.html

http://www.mayoclinic.com/health/scleroderma/DS00362/DSECTION=tests-and-diagnosis

About Charlene Collins
Charlene specializes in health/wellness writing and also in diseases and conditions writing. Charlene wears many hats, she is Christian, mother, friend, cook, writer and ghostwriter.

Scleroderma - What Is It?

Scleroderma - What Is It?

http://sparkpeople.com/resource/health_a-z_detail.asp?AZ=412

What Is It?
Scleroderma is a poorly understood illness that causes widespread hardening of the skin, especially on the hands and face. It also can damage the lungs, heart, kidneys, digestive tract, muscles and joints. It is a long-lasting (chronic) autoimmune disorder, an illness in which the body's immune defenses mistakenly attack the body's own cells rather than protecting them from outside invaders. Scleroderma also is called progressive systemic sclerosis.

There are two types of scleroderma. In the limited form, also called limited systemic sclerosis, the skin is the primary target. In the diffuse form (diffuse systemic sclerosis), the damage not only affects the skin, but also can affect the lungs, kidneys and other internal organs.

In people with scleroderma, scientists have identified abnormal immune proteins called autoantibodies, which are programmed to attack specific components of body cells. They also have found abnormal accumulations of protective T cells (white blood cells that are part of the immune system) in the skin and elsewhere. Although scientists don't understand exactly what happens, they believe that the immune system, perhaps involving these autoantibodies or T cells, somehow damages the body's smallest arteries, called arterioles. These damaged arterioles leak fluid, which causes swelling. They also release chemical factors that stimulate cells called fibroblasts to produce too much collagen, a fibrous protein. In the skin, this leads to thickening, hardening and tightness. Elsewhere in the body, the autoimmune attack of scleroderma can damage the digestive tract, the linings of joints, the outside sheaths of tendons, muscles (including the heart muscle), portions of the heart that regulate heart rhythm, the small blood vessels and the kidney.

Scleroderma is rare, affecting about 14 in every 1 million people worldwide, and it is most common in women aged 35 to 54. The cause is unknown. For some reason, cells called fibroblasts make too much scar-type tissue in the skin and in organs throughout the body. A number of theories have been proposed to explain this, including abnormalities in blood vessel function, abnormal proteins and antibodies in the circulation, and abnormal amounts of chemical messengers instructing fibroblasts to become overly active. Because scleroderma is more common in women during the childbearing years, researchers have looked for a pregnancy-related factor to explain why scleroderma develops. One theory suggests that leftover fetal cells can still be circulating in the mother's bloodstream decades after pregnancy, and may play some role in triggering the autoimmune changes behind scleroderma. Genetic factors and infectious triggers have also been proposed.

Older studies have linked scleroderma to exposure to certain chemicals, specifically vinyl chloride, epoxy resins and aromatic hydrocarbons. Some people who took tryptophan, an amino acid that used to be sold as a dietary supplement, developed a condition similar to scleroderma called eosinophilia myalgia syndrome. Since tryptophan was removed from the market, no further cases of eosinophilia myalgia syndrome have been reported, but the clear link between tryptophan and eosinophilia myalgia syndrome raises the possibility that exposure to something in the environment could trigger scleroderma.

Very Good Article on Symptoms of Scleroderma

Arthritis & Other Rheumatic Diseases

http://www.rush.edu/rumc/page-1098987311761.html


Scleroderma

What is scleroderma?

Scleroderma, also called systemic sclerosis, is a chronic, degenerative disease that affects the joints, skin, and internal organs. Scleroderma is also associated with blood vessel abnormalities.

Scleroderma is considered to be a multifactorial condition. Multifactorial inheritance means that "many factors" are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender (either males or females) is affected more frequently than the other in multifactorial traits. Females are affected with scleroderma three to four times more often than males.

What are the symptoms of scleroderma?

Scleroderma can lead to scarring of the skin, joints, and other internal organs. The following are the most common symptoms of scleroderma. However each individual may experience symptoms differently. Symptoms may include:

thickening and swelling of the tips of the fingers

pale and tingly fingers that may become numb when exposed to cold or when emotionally upset (called Raynaud's phenomenon)

joint pain

taut, shiny, darker skin on large areas such as the face, that may hinder movement
appearance of spider veins

calcium bumps on the fingers or other bony areas

grating noise as inflamed tissues move

frozen (immobile) fingers, wrists, or elbows due to scarring of the skin

sores on fingertips and knuckles

scarring of the esophagus, leading to heartburn and difficulty swallowing

scarring of the lungs, leading to shortness of breath

heart failure and abnormal heart rhythms

kidney disease
The symptoms of scleroderma may resemble other medical conditions or problems. Always consult your physician for a diagnosis.

How is scleroderma diagnosed?

In addition to a complete medical history and physical examination, a diagnosis of scleroderma is usually based on the changes in the skin and internal organs. An antibody test may help distinguish the type of scleroderma present.

Treatment for scleroderma:

Specific treatment for scleroderma will be determined by your physician based on:

your age, overall health, and medical history
extent of the condition
your tolerance for specific medications, procedures, and therapies
expectation for the course of the disease
your opinion or preference
Treatment may include:

nonsteroidal anti-inflammatory medications or corticosteroids (to relieve pain)
penicillamine (to slow the skin thickening process and delay damage to internal organs)
immunosuppressive medications
treating specific symptoms, such as heartburn and Raynaud's phenomenon
physical therapy and exercise (to maintain muscle strength)
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