Scleroderma

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at March 30, 2016
StartDiseasesScleroderma

Scleroderma is identified as the hardening and tightening of the skin. Depending on the severity of scleroderma, it can affect both the skin and the connective tissues, causing damage to the blood vessels, internal organs, and potentially the gastrointestinal tract. Scleroderma is treated through medication, therapy, and in serious circumstances, surgery.

Contents

Definition & Facts

Scleroderma is a group of diseases that affect the skin and the skin's tissues and is most commonly seen in women between the ages of 30 and 50. Although it is a rare disease, scleroderma can go beyond causing damage to the skin and also hurt the blood vessels, internal organs, and the digestive tract. The majority of sufferers from scleroderma will see damage beyond the hardening of only the skin.

There are two kinds of scleroderma, including localized scleroderma and systemic scleroderma. Localized scleroderma does not usually spread beyond the skin and includes the subcategories "morphea" and "linear scleroderma." Morphea is usually identified by varying shapes, sizes, and color of skin while linear scleroderma is a streak or line.

Systematic scleroderma is the more serious form of the disease and includes the subcategories "limited" and "diffuse." Limited occurs in about half of all scleroderma patients, which is slower to spread. It is not uncommon to see localized scleroderma in children, while systematic scleroderma is more common in adults than in children.

Symptoms & Complaints

Because scleroderma first affects the skin, the disease can begin in any part of the body. The first signs of scleroderma is hardened or tightened patches of skin, but the severity, size, and location will vary from person to person and be determined by the type of scleroderma the individual has. The change in skin's texture may make it difficult to move that area of the body.

Scleroderma can also cause the individual to experience what is called Raynaud's phenomenon, which is classified as numbness or pain in the fingers and toes in response to cold temperatures. If scleroderma reaches the digestive system, it can make it difficult for the body to move food through the necessary organs for proper nutrient absorption. This can cause the individual to experience things like acid reflux and malnutrition.

Finally, in the most serious of circumstances, scleroderma can reach the heart, lungs, and kidneys. The symptoms associated to damage to the heart, lungs, or kidneys will vary by situation and level of damage, but once the disease reaches these essential organs, it can become life-threatening.

Causes

The cause of scleroderma is still widely misunderstood, but doctors do know it occurs because of an increased production of collagen. While collagen is something the skin needs in order to remain strong, when there is too much collagen present, the collagen hardens too much. Doctors also understand that the body's immune system essentially turns against itself in producing too much collagen. Doctors and researchers are still working to determine the exact cause of scleroderma and what prompts the body's immune system to product collagen at such high levels.

Those with a higher risk for developing scleroderma are women, but it can still appear in men. It is also more likely that Native Americans or African-Americans develop more serious forms of scleroderma that affect internal organs than those of European descent.

Diagnosis & Tests

Each person's relationship with scleroderma will be different, making it difficult to have a list of symptoms or diagnostic tests for determining if an individual has scleroderma. For an individual who has experienced hardening or tightening of the skin, a doctor's first steps will usually involve performing a complete physical examination to determine if there might be another cause for the change in skin texture.

The doctor may also perform a blood test to determine if the body has an increased amount of antibodies, which could indicate it is trying to fight of infection or disease. Another test that may be performed includes getting a biopsy of the infected skin area. If the scleroderma is believed to have already spread to the internal organs, it might be necessary to undergo a number of breathing tests or have a CT scan of the lungs.

Treatment & Therapy

Unfortunately, there is currently no cure for scleroderma, although the symptoms can be reduced through medications. In most circumstances, skin conditions will fade on their own, but if the disease reaches the internal organs, symptoms may get worse. Drugs prescribed to individuals with scleroderma includes drugs that will dilate blood vessels, suppress the immune system, reduce acid in the stomach, prevent infections, or relieve pain.

Various forms of therapy are also available to help mange pain or return movement in the areas affected by hardened or tightened skin. In the more serious cases of scleroderma, surgery may be necessary. Common surgeries associated with the disease includes lung transplantation if the disease has spread to the internal organs, or amputation.

The symptoms of scleroderma can also be reduced from alternative medicine or lifestyle treatments, including regular exercise, quitting smoking, managing heartburn, and not exposing fingers and toes to the cold without adequate protection.

Those with scleroderma should also practice mediation and relaxation techniques that can reduce stress and relieve pain. In many circumstances a sufferer of scleroderma may be referred to a rheumatologist, or an arthritis specialist, or a dermatologist, or skin specialist, for further examination or tests.

Prevention & Prophylaxis

Without an understanding of why the body begins to develop too much collagen, doctors cannot know what to do to reduce the risk of developing the disease. It is important to note that scleroderma is not a contagious disease and is usually not genetic either.