Scleroderma

background

Scleroderma is a group of disorders that cause the skin and the fibers that provide support and framework for the body (called connective tissues) to harden and tighten. It typically starts with a few dry patches of skin on the face and hands. Over time, these patches of skin become thicker and harder, and more and more skin becomes affected. In some cases, the condition may also involve the internal organs and blood vessels.
Scleroderma is classified into two major categories: localized scleroderma and systemic scleroderma. Localized scleroderma only affects the skin. Often, localized conditions improve or go away on their own over time. However, the skin changes and damage that occurred when the disease was active are permanent.
Systemic scleroderma is a more serious type of scleroderma that affects the skin, deep tissues below the skin, blood vessels, and internal organs. When the internal organs become hard, they no longer function properly and death may occur. Systemic scleroderma may develop suddenly or it may start with the skin and progress gradually to the internal organs.
Scleroderma is a type of autoimmune disorder because it occurs when a patient's immune system does not function properly. Normally, the immune system helps fight against diseases and infections. In scleroderma patients, the immune system attacks the person's own cells. However, researchers have not discovered what causes this abnormal immune response.
Researchers also know that scleroderma is not a contagious illness.
For unknown reasons, scleroderma is more common in women than men. It is also more common in adults than children.
There is currently no cure for scleroderma. Patients who only have skin involvement have a better outlook, but they often suffer from low self-esteem and difficulty performing everyday tasks. The average life expectancy after systemic scleroderma is diagnosed is 12 years. This is because the condition usually worsens over time. Patients who have systemic scleroderma are most likely to die as a result of heart, kidney, lung, or gastrointestinal (digestive) complications.

Related Terms

Calcinosis, CREST, cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, hardened skin, limited cutaneous systemic sclerosis, linear scleroderma, localized scleroderma, morphea scleroderma, overlap syndromes, Raynaud's phenomenon, sine scleroderma, skin disorder, systemic scleroderma, systemic sclerosis, telangiectasia, UCTD, undifferentiated connective tissue disease.