Diseases Home > Systemic Scleroderma

Systemic scleroderma can affect the entire body, but usually affects the skin and tissues beneath it that lead to the blood vessels and major organs. There are three subtypes: limited, diffuse, and sine. Associated symptoms vary in type and intensity. Many people with systemic scleroderma have symptoms that doctors refer to as CREST, which stands for calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias.

What Is Systemic Scleroderma?

Systemic scleroderma (also known as systemic sclerosis) is the term for scleroderma that affects the skin and the tissues beneath skin that lead to the blood vessels and major organs. Systemic scleroderma can affect the entire body, and is typically broken down into three subtypes, which are:
  • Limited
  • Diffuse
  • Sine.
People with systemic sclerosis often have all or some of the symptoms that doctors call CREST, which stands for the following:
(Click Scleroderma Symptoms for more information on CREST and its symptoms.)

Subtypes of Systemic Scleroderma: Limited

Limited scleroderma typically comes on gradually and affects the skin only in certain areas, which include:
  • Fingers
  • Hands
  • Face
  • Lower arms
  • Legs.
Many people with limited scleroderma have Raynaud's phenomenon (a disorder that affects the blood vessels in the fingers, toes, ears, and nose) for years before skin thickening starts. Other people with limited scleroderma start out with skin problems over much of the body, which can improve over time, leaving only the face and hands with tight, thickened skin. Telangiectasias (small enlarged blood vessels near the surface of the skin) and calcinosis (small white lumps that form under the skin) often follow. Raynaud's phenomenon, telangiectasias, and calcinosis are the predominant CREST symptoms in limited scleroderma.

(Click CREST Syndrome for more information about this disease.)
Written by/reviewed by:
Last reviewed by: Arthur Schoenstadt, MD
Last updated/reviewed:
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