Scleroderma is an autoimmune connective tissue disorder that causes excess collagen to form. People with scleroderma may develop thickened hard skin anywhere on their body. The skin appears shiny, loses hair and may darken. It is especially difficult to have scleroderma on one’s face or hands, two of the more common sites, as the condition is so visible for others to see.
Approximately 300,000 people have scleroderma here in the U.S. and it affects women three times as often as men. The cause is unknown. It can appear at any age but frequently develops in people between 30 to 50 years old. Scleroderma is not contagious though studies indicate there may be some genetic influence.
There are two forms of scleroderma: localized and systemic. Localized scleroderma primarily affects the skin in various areas of the body but may affect muscles and bone. It usually does not progress to become systemic. The two types of localized scleroderma are: morphea and linear. Morphea scleroderma appears as localized hardened patches. Linear scleroderma occurs in a single extremity, usually a leg, and can appear as long hardened strip. It can also affect bone growth in children.
Systemic scleroderma can be more serious. It affects internal organs such as the heart, lungs or kidneys as well as blood vessels and the digestive system as thick scar tissue develops and hinders their functioning. Systemic scleroderma is further divided into the groups of: limited and diffuse. The five most common areas affected in limited scleroderma are known by the acronym CREST.
● C- Calcinosis is when calcium deposits form in connective tissue typically on the hands, fingers, face and trunk.
● R- Raynaud’s phenomenon is a condition where blood vessels constrict in the hands and feet in response to cold or stress. Feet and fingers turn white and cold, then turn blue eventually turning red as blood flow resumes.
● E –Esophageal dysfunction due to scarring can lead to swallowing difficulties or chronic heartburn.
● S- Sclerodactyly is tightening of the skin of the fingers due to excess collagen production making it hard to bend or straighten them.
● T –Telangiectasia are red spots that may appear on the face, lips, hands and forearms caused by swelling of tiny blood vessels or capillaries.
Diffuse scleroderma typically comes on suddenly and those patients are more at risk of developing serious organ complications of the disease
Treatment:
Usually a rheumatologist oversees and coordinates the other specialties needed as scleroderma affects numerous parts of the body. Skin thickening may be treated with the rheumatoid drug penicillamine which is thought to decrease collagen production. If other organs are affected by scarring of the tissue, alternative medications can be tried to control symptoms.
Scleroderma affects people differently and many people live with the condition with adjustments to their life but without progression of the disease. Others do suffer from multiple organ problems and battle to regain a balanced life. Patients who seek close monitoring and care during the first three years of skin involvement have the best outlook for the future as the risk of organ complications goes down after that period.
Sources:
www.niams.nih.gov/Health_Info/Scleroderma/default.asp
www.healthscout.com/ency/68/311/main.html
www.medicinenet.com/scleroderma/article.htm
Michele is an R.N. freelance writer with a special interest in woman’s healthcare and quality of care issues. Other articles by Michele can be read at http://www.helium.com/users/487540/show_articles