An Introduction to Scleroderma
Scleroderma is a disease in which the skin becomes progressively hard and thickened. This occurs when immune cells activate, producing scar tissue in the skin, internal organs, and small blood vessels.
Women are three times more likely than men to develop scleroderma. It appears to be more common among African American women than other races, and a woman's risk of developing scleroderma is 15 times greater during her childbearing years.
For some people, scleroderma (particularly the localized forms) is fairly mild and resolves with time. However, some people find that
living with scleroderma has a significant impact on their quality of life.
Scleroderma is derived from the Greek words "sklerosis," meaning hardness, and "derma," meaning skin. Therefore, it literally means "hard skin." Although it is often referred to as a single disease, it is really a symptom of a group of diseases. This group involves the abnormal growth of connective tissue, which supports the skin and internal organs. Therefore, scleroderma is sometimes used as an umbrella term for these disorders.
In some forms of scleroderma, hard, tight skin is the extent of this abnormal process. However, in other forms, the problem goes much deeper, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.
Scleroderma is considered a rheumatic disease and a connective tissue disease. A rheumatic disease refers to a group of conditions characterized by inflammation and/or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects the major substances in the skin, tendons, and bones.
Subtypes of localized scleroderma include morphea and linear scleroderma. Subtypes of systemic scleroderma include limited scleroderma, diffuse scleroderma, and sine scleroderma.
(Click Types of Scleroderma for more information.)
Although scientists do not know the exact cause of scleroderma, they are certain that people cannot catch it from or transmit it to others. Studies also show that it is not inherited.
Scientists suspect that it comes from several factors, which may include:
- Abnormal immune or inflammatory activity
- Genetics
- Environment
- Hormones.
(Click Scleroderma Causes for more information about factors that may play a role in causing this disease.)
Symptoms will depend on the type and subtype of the condition that a person has. However, in most patients, early symptoms are swelling and puffiness of the fingers or hands and
Raynaud's phenomenon (a disorder that affects the blood vessels in the fingers, toes, ears, and nose).
Other symptoms can include:
- Skin ulcers on the fingers
- Joint stiffness in the hands
- Pain
- Sore throat
- Diarrhea.
Your doctor will be able to make a diagnosis if you have typical symptoms and rapid skin thickening. However, a diagnosis may take months, or even years, if your disease only shows some symptoms and your doctor is able to rule out other potential causes. In some cases, a diagnosis is never made because the symptoms that prompted the visit to the doctor go away on their own.
Currently, there are no treatments that control or stop the underlying problem -- the overproduction of collagen. Thus, treatment and management focus on relieving symptoms and limiting damage. Your treatment will depend on the particular problems you are having and will be prescribed or given by your physician. Other treatments include things you can do on your own.
Although no cure for scleroderma currently exists, timely intervention can improve the quality of life.
Although scleroderma is more common in women, it also occurs in men and children, and it affects people of all races and ethnic groups.
However, it often follows patterns based on the specific type of disease. For example:
- Localized forms of scleroderma are more common in people of European descent than in African Americans.
- Morphea usually appears between the ages of 20 and 40.
- Linear scleroderma usually occurs in children or teenagers.
- Systemic scleroderma, whether limited or diffuse, typically occurs in people from 30 to 50 years old. It affects more women of African American than European descent.
Scleroderma can be hard to diagnose because it can occur in conjunction with or resemble other diseases.
Scientists can only estimate how many cases of the disease there actually are. In the United States, the number of people with systemic sclerosis ranges from 40,000 to 165,000. By contrast, a survey that included all scleroderma-related disorders (including
Raynaud's phenomenon) suggested a number between 250,000 and 992,500.
