The list of types of Scleroderma mentioned in various sources includes:
The group of diseases we call scleroderma falls into two
main classes: localized scleroderma and systemic sclerosis. (Localized
diseases affect only certain parts of the body; systemic diseases can
affect the whole body.) Both groups include subgroups. (See chart.)
Although there are different ways these groups and subgroups may be
broken down or referred to (and your doctor may use different terms from
what you see here), the following is a common way of classifying these
diseases:
Localized Scleroderma
Localized types of scleroderma are those limited to the skin and related tissues and, in some cases, the muscle below. Internal organs are not affected by localized scleroderma, and localized scleroderma can never progress to the systemic form of the disease. Often, localized conditions improve or go away on their own over time, but the skin changes and damage that occur when the disease is active can be permanent. For some people, localized scleroderma is serious and disabling.
There are two generally recognized types of localized scleroderma:
Morphea: Morphea (mor-FEE-ah) comes from a Greek word that means "form" or "structure." The word refers to local patches of scleroderma. The first signs of the disease are reddish patches of skin that thicken into firm, oval-shaped areas. The center of each patch becomes ivory colored with violet borders. These patches sweat very little and have little hair growth. Patches appear most often on the chest, stomach, and back. Sometimes they appear on the face, arms, and legs.
Morphea can be either localized or generalized. Localized morphea limits itself to one or several patches, ranging in size from a half-inch to 12 inches in diameter. The condition sometimes appears on areas treated by radiation therapy. Some people have both morphea and linear scleroderma (described below). The disease is referred to as generalized morphea when the skin patches become very hard and dark and spread over larger areas of the body.
Regardless of the type, morphea generally fades out in 3 to 5 years; however, people are often left with darkened skin patches and, in rare cases, muscle weakness.
Linear scleroderma: As suggested by its name, the disease has a single line or band of thickened and/or abnormally colored skin. Usually, the line runs down an arm or leg, but in some people it runs down the forehead. People sometimes use the French term en coup de sabre, or "sword stroke," to describe this highly visible line.
Systemic Scleroderma (also known as Systemic Sclerosis)
Systemic scleroderma, or systemic sclerosis, is the term for the disease that not only includes the skin, but also involves the tissues beneath to the blood vessels and major organs. Systemic sclerosis is typically broken down into diffuse and limited disease. People with systemic sclerosis often have all or some of the symptoms that some doctors call CREST, which stands for the following:
Limited scleroderma: Limited scleroderma typically comes on gradually and affects the skin only in certain areas: the fingers, hands, face, lower arms, and legs. Many people with limited disease have Raynaud's phenomenon for years before skin thickening starts. Others start out with skin problems over much of the body, which improves over time, leaving only the face and hands with tight, thickened skin. Telangiectasias and calcinosis often follow. Because of the predominance of CREST in people with limited disease, some doctors refer to limited disease as the CREST syndrome.
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). Some people may have more area of their skin affected than others. Internally, it can damage key organs such as the heart, lungs, and kidneys.
People with diffuse disease are often tired, lose appetite and weight, and have joint swelling and/or pain. Skin changes can cause the skin to swell, appear shiny, and feel tight and itchy.
The damage of diffuse scleroderma typically occurs over a few years. After the first 3 to 5 years, people with diffuse disease often enter a stable phase lasting for varying lengths of time. During this phase, skin thickness and appearance stay about the same. Damage to internal organs progresses little, if at all. Symptoms also subside: joint pain eases, fatigue lessens, and appetite returns.
Gradually, however, the skin starts to change again. Less collagen is made and the body seems to get rid of the 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. Some patients' skin returns to a somewhat normal state, while other patients are left with thin, fragile skin without hair or sweat glands. More serious damage to 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 with diffuse disease develop these problems. Early diagnosis and continual and careful monitoring are important.
Sine scleroderma: Some doctors break systemic sclerosis down into a third subset called systemic sclerosis sine (SEEN-ay, Latin for "without") scleroderma. Sine may resemble either limited or diffuse systemic sclerosis, causing changes in the lungs, kidneys, and blood vessels. However, there is one key difference between sine and other forms of systemic sclerosis: it does not affect the skin. (Source: excerpt from Handout on Health Scleroderma: NIAMS)
More general medical disease topics related to Scleroderma include:
What do you think about the features of this website? Take our user survey and have your say:
Next articles:
Tools & Services:
Medical Articles:
Search Specialists by State and City
By using this site you agree to our Terms of Use. Information provided on this site is for informational purposes only; it is not intended as a substitute for advice from your own medical team. The information on this site is not to be used for diagnosing or treating any health concerns you may have - please contact your physician or health care professional for all your medical needs. Please see our Terms of Use.
Copyright © 2009 Health Grades Inc. All rights reserved.
Whenever you go to a hospital or clinic for a major procedure or diagnostic test, one of the many forms you are given to sign is an "informed...
Germs are a fact of life and catching an infectious disease like a cold may seem inevitable. But there are simple ways to protect yourself against...
Sexual contact can sometimes result in problems. An unwanted pregnancy or sexually transmitted diseases may be some of those consequences. But by...
Sleep is necessary to feel refreshed, but now we know sleep actually impacts the way the body functions. Sleeping poorly can affect how often you get...