Understanding the Subtypes of Systemic Scleroderma

Types of Systemic Scleroderma

Systemic sclerosis is typically broken down into the following subtypes, each with its own symptoms:
  • Limited
  • Diffuse
  • Sine.
Limited Scleroderma Symptoms
Symptoms of limited scleroderma typically come on gradually and affect 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 the condition start out with skin problems over much of the body, which improve over time, leaving only the face and hands with tight, thickened skin. Telangiectasias and calcinosis often follow. As mentioned, Raynaud's phenomenon, telangiectasias, and calcinosis are the predominant CREST symptoms in limited scleroderma.

(Click CREST Syndrome for more information about this disease.)
Diffuse Scleroderma Symptoms
Diffuse scleroderma symptoms typically come 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 of diffuse scleroderma typically occurs over a few years.
After the first three to five years, people with diffuse scleroderma 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. Diffuse scleroderma symptoms also subside, which means that joint pain eases, fatigue lessens, and appetite returns.
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 people with diffuse scleroderma develop these problems. Early diagnosis and continual and careful monitoring are important factors in this.

Scleroderma Disease

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