Scleroderma: Both a Rheumatic Disease and a Connective Tissue Disease
Scleroderma literally means hard skin. Derived from the Greek words "sklerosis," meaning hardness, and "derma," meaning skin.
Though it is often referred to as if it were a single disease, scleroderma is really a symptom of a group of diseases that involve the abnormal growth of connective tissue, which supports the skin and internal organs. Scleroderma is sometimes used, therefore, as an umbrella term for these disorders.
In some forms of scleroderma, hard, tight skin is the extent of this abnormal process. In other forms, however, the problem goes much deeper, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.
Scleroderma is called both a rheumatic disease and a connective tissue disease.
Although scleroderma is more common in women, the disease also occurs in men and children. It affects people of all races and ethnic groups. However, there are some patterns by disease type. For example:
Because scleroderma can be hard to diagnose and it overlaps with or resembles other diseases, scientists can only estimate the actual number of cases.
For some people, scleroderma is fairly mild and resolves with time. But for others, living with the disease and its effects day to day has a significant impact on their quality of life.
Although scientists don't know exactly what causes scleroderma, they are certain that people cannot catch it from or transmit it to others. Studies of twins suggest it is also not inherited.
Scientists suspect that scleroderma comes from several factors that may include:
What Are the Different Types of Scleroderma?
Scleroderma falls into two main classes: localized scleroderma and systemic sclerosis.
Both groups include subgroups. There are different ways these groups and subgroups may be broken down. (your doctor may even use different terms)
Localized diseases affect only certain parts of the body; whereas systemic diseases can affect the whole body.
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 and Linear Scleroderma
Morphea: Morphea 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.
