The name scleroderma comes from the Greek words “sklerosis,” for hardness and “derma” for skin. Scleroderma is part of a group of connective tissue disorders. For unknown reasons, people with scleroderma develop an immune reaction where their body overproduces collagen, causing hardening of the skin and stiffness in joints, muscles and tendons. Some forms of scleroderma cause this hardening to also occur in the blood vessels and internal organs such as the heart, lung or kidneys.
Scleroderma is divided into two basic types: localized and systemic. Localized scleroderma affects the skin and tissues but does not go into the deeper organs. Examples of this type of scleroderma are linear scleroderma, which is a hardened band of skin usually on an arm or leg and morphea, which appears as thickened ivory colored patches that develop on various parts of the body. Localized scleroderma may improve or go away on its own but will not spread and develop into systemic scleroderma.
Systemic scleroderma is more involved and affects the tissues, blood vessels and organs in addition to the skin. There are two main types of this form of scleroderma: limited cutaneous scleroderma and diffuse cutaneous scleroderma.
• Limited cutaneous scleroderma affects only particular parts the body such as the fingers or legs then gradually skin hardening occurs later even though other common accompanying symptoms such as Raynaud’s disease may have been evident for some time. Raynaud’s disease, a related immune disease, causes contraction of blood vessels in response to cold so that those parts of the body become white then bluish in color.
• Diffuse cutaneous scleroderma in contrast comes on rapidly. Skin thickening changes begin occurring all over the body and internal organs are also affected simultaneously. People experience loss of appetite, joint swelling and the skin appears shiny and tight. People with this form of scleroderma do tend to stabilize after three to five years and their skin may soften, their appetite improve and their joint pain ease.
Treatments:
Scleroderma occurs in people between the ages of 30 to 50 affecting more women than men. There is no cure but there are treatments that may reduce the pain and other complications of the disease. Certain drugs treat scleroderma directly such as corticosteroids, immune suppressing medications and NSAIDS (non-steroid anti-inflammatories). Other drugs are used to treat high blood pressure, kidney problems, gastric upsets such as heartburn and difficulty breathing. Therapies that use UV light may help with skin hardening and physical therapy can assist in managing pain.
Support organizations such as www.scleroderma.org can provide physician referrals and access to support group. Other support groups can be searched on line by entering the state you live in followed by the word "scleroderma".
sources:
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001465/
http://www.scleroderma.org/medical/overview.shtm
http://www.mayoclinic.com/health/scleroderma/DS00362
http://www.niams.nih.gov/Health_Info/Scleroderma/
Edited by Alison Stanton
Michele is an R.N. freelance writer with a special interest in woman’s health care and quality of care issues. Other articles by Michele are at www.helium.com/users/487540/show_articles