Diseases Home > Limited Scleroderma and Diffuse Scleroderma

Limited Scleroderma
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. Some doctors refer to limited scleroderma as CREST syndrome because of the predominance of CREST symptoms.
Diffuse Scleroderma
Diffuse scleroderma typically comes on suddenly. Skin thickening occurs quickly and over much of the body, affecting the hands, face, upper arms, upper legs, chest, and stomach in a symmetrical fashion (for example, if one arm or one side of the trunk is affected, the other is also affected). Scleroderma can also damage internal key organs, such as the heart, lungs, and kidneys.
People with diffuse scleroderma often experience:
  • Tiredness
  • Loss of appetite and weight
  • Joint swelling and/or pain
  • Skin changes such as swelling, appearing shiny, and feeling tight and itchy.
The damage caused by diffuse scleroderma typically occurs over a few years. After the first three to five years, people often enter a stable phase lasting for varying lengths of time. During this phase, skin thickness and appearance stay about the same and damage to internal organs progresses little, if at all. Symptoms also subside, which means that joint pain eases, fatigue lessens, and appetite returns.

(Click Scleroderma Symptoms for more information about the various symptoms associated with this form of the disease.)
The skin will gradually begin to change. Less collagen will be made, and the body will get rid of excess collagen. This process, called "softening," tends to occur in reverse order of the thickening process: the last areas thickened are the first to begin softening.
For some people, skin returns to normal, while other people are left with thin, fragile skin without hair or sweat glands. More serious damage to the heart, lungs, or kidneys is unlikely to occur, unless previous damage leads to more advanced deterioration.
People with diffuse scleroderma face the most serious long-term outlook if they develop severe kidney, lung, digestive, or heart problems. Fortunately, less than one-third of patients develop these problems. Early diagnosis and continual and careful monitoring are important factors in this.
Written by/reviewed by:
Last reviewed by: Arthur Schoenstadt, MD
Last updated/reviewed:
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