Scleroderma means hard skin but the hardness is not limited to the skin – the internal organs and their blood supply may become damaged. The most characteristic feature of scleroderma is the build-up of tough scar-like fibrous tissue in the skin. Scleroderma is not a single disease, but rather a symptom of a group of diseases complicated by the abnormal growth of connective tissue which supports the skin and internal organs. Although scleroderma is more common in women, the disease also occurs in men and children. It affects people of all races and ethnic groups. However, there are some patterns by disease type. Scleroderma is a relatively uncommon problem affecting only 200 to 300 people per million in the U.S. Some 12 to 20 new cases per million are diagnosed annually.
The cause of scleroderma is unknown. Like many other rheumatic disorders, scleroderma is believed to be an autoimmune disease. An autoimmune disease is one in which the immune system, for unknown reasons, turns against ones own body. Although the cause is unknown, it is known that the body produces too much of a protein called collagen. Excess collagen is deposited causing thickening and hardening. Scleroderma is not believed to be contagious; you cannot get it by hugging or kissing someone or other intimate contact. There is an increased incidence of certain types other autoimmune diseases and cancer among scleroderma patients. Preliminary research suggests that patients with certain gene variations, or genetic polymorphisms, may be more susceptible to scleroderma than those who do not carry the polymorphism.
The skin begins to thicken and harden on the hands, feet, and face. The fingers may swell. Patients with diffuse scleroderma may have whole hand swelling before the skin significantly thickens.
Common symptoms are-
· Muscle pain and weakness
· Dry eyes and mouth
* Difficulty swallowing
* Poor absorption of nutrients, weight loss
* Liver blockage, jaundice
* Shortness of breath
* Abnormal heart rhythms
* Heart failure
* High blood pressure
* Kidney failure
Aggressive treatment of elevations in blood pressure have been extremely important in prevent kidney failure. Blood pressure medications, such as captopril, are frequently used.
As each person with scleroderma is affected in a different way, other treatments are used to treat problems if or when they occur. For example, there are acid-suppressant drugs for acid reflux and gullet inflammation, anti-hypertensive drugs for high blood pressure and anti-inflammatory drugs for lung inflammation.
For patients with weakness and muscle disease associated with scleroderma, glucocorticoids (such as prednisone) and/or immunosuppressive medications may be effective treatments.
Much research is ongoing into new treatments for scleroderma. Patients and their families should know that experts remain optimistic and work towards a cure will continue.