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Scleroderma / Sclerosis, Systemic

Definition :
Scleroderma (sklere-o-DER-muh) is a rare, progressive disease that leads to hardening and tightening of the skin and connective tissues — the fibers that provide the framework and support for your body. Scleroderma usually starts with a few dry patches of skin on the hands or face that begin getting thicker and harder. These patches then spread to other areas of the skin. In fact, scleroderma literally means "hard skin."

In some cases, scleroderma also affects the blood vessels and internal organs. Scleroderma is one of a group of arthritic conditions called connective tissue disorders. In these disorders, a person's antibodies are directed against his or her own tissues.

Researchers haven't established a definitive cause for scleroderma. It's more common in women than in men and more common in adults than in children. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease. Scleroderma isn't considered contagious or cancerous, but this chronic condition can greatly affect self-esteem and the ability to accomplish everyday tasks.

Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Scleroderma results from an overproduction and accumulation of collagen in body tissues.

Although doctors aren't sure what prompts this abnormal collagen production, the body's immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen. In addition to its effects on your skin, some types of scleroderma affect tiny blood vessels and can affect almost every organ.

Types of scleroderma
Doctors classify scleroderma into different subsets :

Localized scleroderma
This type of scleroderma is limited to your skin and the deep tissues below your skin. It includes the following subclassifications :

  • Morphea. In this form, oval-shaped thick patches appear on your skin — white in the middle, with a purple border. These patches are most likely to occur on your torso, but they can also appear on your arms, legs or forehead.
  • Linear scleroderma. This form results in bands or streaks of hardened skin on one or both of your arms or legs, or on your forehead.

Systemic scleroderma
This type of scleroderma affects not only your skin but also your blood vessels and major organs. It's also called systemic sclerosis and includes the following subclassifications :

  • Diffuse cutaneous systemic sclerosis. This type affects the skin of your fingers, hands, arms, legs, face, neck and trunk. It can also affect internal organs, such as your lungs, heart, kidneys and gastrointestinal tract — including your esophagus. It can hinder the functions of your digestive system, create respiratory problems and cause kidney failure. When left untreated, systemic scleroderma may be fatal within several years of onset.
  • Limited cutaneous systemic sclerosis. This type involves the skin of your fingers, lower arms and legs, face, and neck. A variation is called CREST syndrome.
  • Sine scleroderma. Some doctors may describe one variation of systemic scleroderma as sine scleroderma, which can be similar to either limited or diffuse scleroderma, the difference being that this form doesn't affect your skin.

Overlap syndrome
This is diffuse or limited systemic sclerosis with features of one or more of the other connective tissue diseases.

Undifferentiated connective tissue disease
This has features of systemic sclerosis, but there are no clinical or specific laboratory findings to make a definite diagnosis.

When to seek medical advice :
If you're experiencing the early signs and symptoms of scleroderma — numbness, pain or color changes in the skin of your extremities, gradual hardening and tightening of your skin, and stiffness or pain in your joints — see your doctor. It's important to establish a diagnosis early before the disease progresses and to determine whether the condition is affecting your internal organs.

In addition to thickening and hardening of your skin, scleroderma can cause your skin to lose its elasticity and become shiny as it stretches across underlying bone. Other signs and symptoms may include :
  • Numbness, pain or color changes in your fingers, toes, cheeks, nose and ears, often brought on by cold or emotional distress (Raynaud's phenomenon)
  • Stiffness or pain in your joints and curling of your fingers
  • Digestive problems ranging from poor absorption of nutrients to delayed movement of food due to impaired muscular activity in your intestine
  • Sores over joints, such as your elbows and knuckles
  • Puffy hands and feet, particularly in the morning

Scleroderma can be difficult to diagnose. It's rare and, early on, it can affect the skin as well as the joints, making it look like other diseases.

To make a diagnosis, your doctor reviews your medical history and conducts a physical examination. As part of the examination, your doctor looks at your skin, checking for thickened and hardened areas. Your doctor may also examine and touch some of your joints and tendons to check for possible changes in connective tissue beneath your skin. Because Raynaud's phenomenon is commonly a sign of scleroderma, your doctor will likely look for color changes in your skin that may indicate Raynaud's.

Your doctor may also conduct the following tests :

  • Blood tests. People with scleroderma have elevated blood levels of certain antibodies produced by the immune system.
  • Tissue sample. Your doctor may remove a small tissue sample (biopsy) of your affected skin to be examined in the laboratory for abnormalities.

Your doctor may recommend other diagnostic tests to identify any lung, heart or gastrointestinal complications accompanying scleroderma.

Complication :
Having systemic scleroderma may result in a number of other health conditions :

  • Gastrointestinal complications. In scleroderma, wasting occurs in the muscular walls of your intestine. This can reduce absorption of nutrients and movement within the intestine, resulting in weight loss and malnutrition. When scleroderma affects the muscular lining of your esophagus, heartburn can occur.
  • Lung complications. Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).
  • Kidney complications. When scleroderma affects your kidneys, you can develop an elevated blood pressure and an increased level of protein in your urine. More serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
  • Heart complications. Scarring of heart tissue increases your risk of heart arrhythmias and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart (pericarditis).

Scleroderma has no known cure — there's no treatment to stop the overproduction of collagen. Your doctor may recommend a number of medications to make it easier for you to live with scleroderma by treating its symptoms. Your doctor may also suggest medications to prevent complications of scleroderma that may affect various organs. Here are some of the many treatments prescribed for the symptoms and complications of this condition.

Skin changes
If you have localized scleroderma, your doctor may recommend a topical treatment, such as a moisturizer or corticosteroid medication that you apply to your skin. Corticosteroid medications impede your body's ability to make substances that can cause inflammation.

If your condition involves a large area of skin, your doctor may recommend additional treatments. Doctors sometimes prescribe minocycline (Minocin, Dynacin) to control the skin-related (cutaneous) symptoms of scleroderma, although no studies have addressed its long-term effectiveness. In preliminary studies, light therapy (phototherapy) also has proved effective in treating the lesions that are associated with scleroderma, but more research is needed.

Cosmetic treatments are another consideration. Some people with scleroderma are discouraged or embarrassed by lesions and marks on the skin, including tiny dilated blood vessels that often appear on the face (telangiectasia). Specialized brands of foundation makeup and pulsed dye laser surgery can help camouflage or eliminate these lesions. Consult a dermatologist about treatments for skin changes.

Circulation problems
Your doctor may also prescribe medications to dilate blood vessels and promote circulation. These medications can prevent high blood pressure and kidney problems and help treat Raynaud's phenomenon.

Medications that help with blood circulation include :

  • Calcium channel blockers.
  • Alpha blockers
  • Angiotensin-converting enzyme (ACE) inhibitors
  • Angiotensin II receptor blockers
  • Low-dose enteric-coated aspirin

Creams containing nitroglycerin also may help promote circulation.

Joint stiffness, pain and inflammation
Your doctor may prescribe anti-inflammatory medications such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs) or low-dose corticosteroids to relieve joint pain and stiffness.

Often, along with NSAIDs, doctors prescribe certain medications called disease-modifying antirheumatic drugs (DMARDs). These medications seem to do their job by having an effect on immune systems that have gone out of control, but doctors don't understand exactly how DMARDs work. Common DMARDs include :

  • Hydroxychloroquine (Plaquenil). This drug has relatively few side effects, and it's also effective for the arthritis that can be associated with scleroderma. Apart from hydroxychloroquine's apparent ability to affect the way immune cells work, scientists don't completely understand how it helps tame the disease process.
  • Penicillamine (Cuprimine, Depen). Similar to other DMARDs, penicillamine can reduce inflammation. Its full effect may require many months to develop, but its beneficial effects may be longer lasting. However, because of a relatively high incidence of adverse reactions to this drug and studies casting doubt on its effectiveness, its use has declined in recent years.
  • Methotrexate (Rheumatrex, Trexall). This drug does its job by affecting cells that are responsible for some of the pain, inflammation and joint swelling that accompany scleroderma. Trials have shown conflicting results regarding the effectiveness of methotrexate in treating scleroderma.

Immunosuppresents are another class of medications that can help manage out-of-control immune systems. Cyclophosphamide (Cytoxan) is one example. This extremely potent medication works by damaging cells' genetic information. In particular, it kills white blood cells called lymphocytes that are part of autoimmune disease.

Lung damage
If you have scleroderma that affects your lungs, you may need additional medications. Cyclophosphamide (Cytoxan) is sometimes used to treat pulmonary fibrosis. A 2006 study of people with scleroderma-related lung disease found cyclophosphamide modestly improved lung function and quality of life. The long-term effects of cyclophosphamide treatment in people with scleroderma are unknown. Bosentan (Tracleer) is an oral medication that has been approved for pulmonary hypertension in people with scleroderma.

Digestive difficulties
If scleroderma has affected your esophagus and you're experiencing heartburn, your doctor may suggest prescription medications that decrease stomach acid production. These medications include H-2-receptor blockers and proton pump inhibitors. Your doctor may also suggest antibiotics, special diets and medications that improve your gut's ability to contract.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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