What Causes Scleroderma?
Although scientists donít 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
Abnormal immune or
inflammatory activity: Like many other
rheumatic disorders, scleroderma is believed to be an
autoimmune disease. An autoimmune disease is one in
which the immune system, for unknown reasons, turns
against oneís own body.
In scleroderma, the immune system is
thought to stimulate cells called fibroblasts so they
produce too much collagen. The collagen forms thick
connective tissue that builds up within the skin and
internal organs and can interfere with their
functioning. Blood vessels and joints can also be
While genes seem to put certain people at risk for
scleroderma and play a role in its course, the disease
is not passed from parent to child like some genetic
Research suggests that exposure to some environmental
factors may trigger sclerodermalike disease (which is
not actually scleroderma) in people who are genetically
predisposed to it. Suspected triggers include viral
infections, certain adhesive and coating materials, and
organic solvents such as vinyl chloride or
trichloroethylene. But no environmental agent has been
shown to cause scleroderma. In the past, some people
believed that silicone breast implants might have been a
factor in developing connective tissue diseases such as
scleroderma. But several studies have not shown evidence
of a connection.
the middle to late childbearing years (ages 30 to 55)
women develop scleroderma 7 to 12 times more often than
men. Because of female predominance at this and all
ages, scientists suspect that hormonal differences
between women and men play a part in the disease.
However, the role of estrogen or other female hormones
has not been proven.
Who Gets Scleroderma?
A lthough 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:
- 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, and 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.
Because scleroderma can be hard to
diagnose and it overlaps with or resembles other
diseases, scientists can only estimate how many cases
there actually are. Estimates for the number of people
in the United States with systemic sclerosis range from
40,000 to 165,000. By contrast, a survey that included
all scleroderma-related disorders, including Raynaudís
phenomenon, suggested a number between 250,000 and
For some people, scleroderma
(particularly the localized forms) 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.
How Is Scleroderma Diagnosed?
Depending on your particular symptoms, a
diagnosis of scleroderma may be made by a general
internist, a dermatologist (a doctor who specializes in
treating diseases of the skin, hair, and nails), an
orthopaedist (a doctor who treats bone and joint
disorders), a pulmonologist (lung specialist), or a
rheumatologist (a doctor specializing in treatment of
musculoskeletal disorders and rheumatic diseases). A
diagnosis of scleroderma is based largely on the medical
history and findings from the physical exam. To make a
diagnosis, your doctor will ask you a lot of questions
about what has happened to you over time and about any
symptoms you may be experiencing. Are you having a
problem with heartburn or swallowing? Are you often
tired or achy? Do your hands turn white in response to
anxiety or cold temperatures?
Once your doctor has taken a thorough
medical history, he or she will perform a physical exam.
Finding one or more of the following factors can help
the doctor diagnose a certain form of scleroderma:
- changed skin appearance and texture, including
swollen fingers and hands and tight skin around the
hands, face, mouth, or elsewhere
- calcium deposits developing under the skin
- changes in the tiny blood vessels (capillaries)
at the base of the fingernails
- thickened skin patches.