What is Scleroderma?
Scleroderma is an autoimmune disease which means that it is a condition in which the body’s immune system attacks its own tissues. The normal role of the immune system is to provide protection from outside invaders such as bacteria and viruses. In autoimmune disorders, this ability to distinguish foreign from self is compromised. As immune cells attack the body’s own tissue, inflammation and damage result. Scleroderma (the name means “hard skin”) can vary a great deal in terms of severity. For some, it is a mild condition; for others it can be life threatening. Although there are medications to slow down disease progression and help with symptoms, right now there is no cure for scleroderma.
Types of Scleroderma
There are two main forms of scleroderma: systemic (systemic sclerosis, SSc) that usually affects the internal organs or internal systems of the body as well as the skin, and localized that affects a local area of skin either in patches (morphea) or in a line down an arm or leg (linear scleroderma), or as a line down the forehead (scleroderma en Coup de Sabre). It is very unusual for localized scleroderma to develop into the systemic form.
Systemic Sclerosis (SSc)
To make matters more confusing, there are two major types of systemic sclerosis or SSc: limited cutaneous SSc and diffuse cutaneous SSc. The difference between limited cutaneous and diffuse cutaneous SSc is the extent of skin involvement. In limited SSc, skin thickening only involves the hands and forearms, lower legs and feet. In diffuse cutaneous disease, the hands, forearms, upper arms, thighs, or trunk are affected. The face can be affected in both forms. The importance of making the distinction between limited and diffuse disease is that the extent of skin involvement tends to reflect the degree of internal organ involvement.
Localized Scleroderma
Localized scleroderma is almost always a purely skin condition and is virtually never associated with the severe and potentially life-threatening complications of SSc.
Quick Questions & Answers
- How serious is scleroderma?
Any chronic disease can be serious. The symptoms of scleroderma vary greatly for each person, and the effects of scleroderma can range from very mild to life threatening. The seriousness will depend on the parts of the body, which are affected, and the extent to which they are affected. A mild case can become more serious if not properly treated. Prompt and proper diagnosis and treatment by qualified physicians may minimize the symptoms of scleroderma and lessen the chance for irreversible damage.
- How is scleroderma diagnosed?
The diagnostic process may require consultation with rheumatologists (autoimmune disease specialists), and/or dermatologists (skin specialists) and require blood studies and numerous other specialized tests depending upon which organs are affected.
- What causes scleroderma?
The exact cause or causes of scleroderma are still unknown, but scientists and medical researchers are working hard to make those determinations. It is known that scleroderma involves an overproduction of collagen.
- Is scleroderma genetic?
Most individuals do not have any relatives with scleroderma and their children do not get scleroderma. Research indicates that there is a susceptibility gene, which raises the likelihood of getting scleroderma, but the gene alone does not cause the disease.
- What is the treatment for scleroderma?
Currently, there is no cure for scleroderma, but there are many treatments available to help particular symptoms. Some treatments are directed at decreasing the activity of the immune system. Some people with mild disease may not need medication at all and occasionally people can go off treatment when their scleroderma is no longer active. Because there is so much variation from one person to another, there is great variation in the treatments prescribed.