Limited systemic scleroderma, also known as limited cutaneous systemic scleroderma, is a subtype of systemic scleroderma, (the other being diffuse scleroderma). It is autoimmune condition marked by skin thickening accompanied by the accumulation of scar tissue in internal organs such as the heart, lungs, and intestinal tract.
Limited vs. diffuse systemic scleroderma
Limited scleroderma is milder and more common than diffuse scleroderma, accounting for about 60 percent of systemic scleroderma cases. It usually develops at a slower pace and may change very little over the years. Organ damage rarely occurs in people with limited scleroderma.
Symptoms of limited scleroderma
Patients with limited scleroderma may find their skin turning hard, thick, and shiny in areas of the face, below the elbows, and below the knees. Like diffuse systemic scleroderma, patients also may experience Raynaud’s phenomenon, a symptom in which fingers turn white, blue, and then red in response to stress or cold environments.
Those with limited scleroderma may experience a collection of symptoms known as the CREST syndrome. The acronym stands for:
- C – calcinosis (calcium deposits under the skin and in tissues)
- R – Raynaud’s phenomenon
- E – esophageal dysfunction
- S – sclerodactyly (thick skin on the fingers)
- T – telangiectasias (enlarged blood vessels that appear as red spots on the skin)
Patients with limited scleroderma also may have damage in the main vessel sending blood into the lungs, leading to raised blood pressure, which is known as pulmonary arterial hypertension (PAH), or lung scarring (fibrosis) known as interstitial lung disease (ILD). Both these conditions, if left untreated, are leading causes of death in those with limited scleroderma.
Diagnosing limited scleroderma
Limited scleroderma is diagnosed based on a score that measures the extent of skin symptoms. Blood tests for specific antibodies that attack the body’s own tissues, known as autoantibodies, and to assess kidney function, also may be done. Chest X-rays and lung CT scans may be performed to determine whether the lungs are damaged.
Treatment and prognosis of limited scleroderma
So far, scleroderma is an incurable condition, but there are several treatment options available to help manage symptoms and prevent associated complications. These may be different for each patient because not everyone has the same symptoms. Generally, those with limited scleroderma tend to fare better than those who have diffuse systemic scleroderma.
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