Scleroderma (Systemic Sclerosis)

What is scleroderma?

Scleroderma, or systemic sclerosis, is a disease affecting the skin and other organs that is one of the autoimmune rheumatic diseases. The primary finding in scleroderma is thickening and tightening of the skin. Effective treatments are available for some forms of the disease, although, scleroderma is not yet curable.

Scleroderma, also known as systemic sclerosis, is a chronic disease that causes skin thickening and tightening, a buildup of scar tissue, and damage to internal body organs. There are several types of scleroderma and related diseases with complications ranging from minor to life‐threatening. Therefore, the terminology can be confusing.

The two broad categories are “localized scleroderma” which indicates distinct skin lesions, and "systemic sclerosis" (scleroderma) which indicates similar skin symptoms and the potential for internal organ disease.

Other diseases affecting the skin that may be confused with scleroderma include scleredema, scleromyxedema, eosinophilic fasciitis, and nephrogenic systemic fibrosis.

What causes scleroderma?

The cause of scleroderma is not known. Genetic factors appear to increase a patient’s chance of getting the disease. However, some data suggests that exposure to industrial solvents or an environmental agent may play a role in leading to scleroderma. Scleroderma‐like syndromes also have been clearly linked to agents as varied as contaminated rapeseed oil, polyvinylchloride, and an impurity in one preparation of L‐tryptophan. That said, the vast majority of patients with scleroderma do not have a history of exposure to any suspicious toxins.

Who gets scleroderma?

Scleroderma is a relatively rare illness affecting only 75,000 – 100,000 people in the United States. Of these, 75% percent are women, usually diagnosed between the ages of 30 and 50 years. Twins and family members of patients with scleroderma or other autoimmune connective tissue diseases, such as lupus, appear to be at a slightly increased risk. Children can get scleroderma, although the pattern and extent of disease may be different for children compared to adults.

Points to remember

• Scleroderma differs from person to person, but can be a very serious disease.
• Physical measures and medications will help control Raynaud's phenomenon and heartburn in many patients.
• Effective treatments are available even for patients with severe disease, including acute kidney disease, pulmonary hypertension, lung inflammation, and gastrointestinal problems.
• The key is to recognize organ involvement early and treat it before irreversible organ damage occurs.
• Patients with scleroderma should be seen by physicians with specialized expertise in the care of this complex disease.