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What Is Scleroderma?
WHAT IS SCLERODERMA
?
Scleroderma is a disease that can affect the
skin, joints, blood vessels, and internal organs. The word scleroderma means
"hard skin." Most people with scleroderma have problems with their skin and
other parts of their bodies.
Scleroderma is a chronic disease. This means it may
last for months, years, or for a lifetime. There is no cure for it, but it
can be treated. With early detection and proper management, many people with
scleroderma can lead full, productive lives.
There are two forms of scleroderma: Localized
and Generalized (also called systemic sclerosis).
LOCALIZED
SCLERODERMA
Localized scleroderma affects mainly the skin.
It can affect skin in different areas of the body. It may also affect muscles
and bone, but it does not affect internal organs. This form is usually not
as severe as generalized scleroderma. People who develop localized scleroderma
usually do not develop generalized scleroderma. There are two types of localized
scleroderma: Morphea and
Linear.
MORPHEA
Morphea happens when hard, oval-shape patches
form on the skin. The patches are usually whitish with a purplish ring around
them. They usually occur on the trunk, but can also occur on the face, arms,
legs, and other parts of the body. Morphea often improves by itself, over
time.
LINEAR
Linear is a line of thickened skin that occurs
in areas such as the arms, legs, or forehead. It can occur in more than one
area. The line can extend deep into the skin and affect the bones and muscles
underneath it. This can affect the motion of joints and muscles, as well
as the growth of the affected area.
When the line forms a long crease on the head or neck,
it is sometimes called en coup de sabre (the strike of the sword).
Linear scleroderma usually occurs in childhood.
GENERALIZED
Generalized scleroderma affects many parts
of the body. It can affect the skin as well as internal body parts, such
as blood vessels, the digestive system (esophagus, stomach, and bowel), the
heart, lungs, kidneys, muscles, and joints. The severity of this form depends
on the organs affected and how much they are affected. In rare cases, scleroderma
may affect only some internal system, leaving the skin and joints
untouched.
There are two types of generalized scleroderma:
Limited (also called the CREST Syndrome) and
Diffuse.
CREST stands for a combination of symptoms:
Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly,
and Telangiectasia. This type usually has a slow onset, with the first symptoms
appearing 10 to 20 years before the full syndrome occurs. It usually affects
the skin on the face, fingers, and hands. Later on, it may affect internal
organs, such as the esophagus (the tube leading from the mouth to the stomach),
the lungs and bowels.
DIFFUSE
Diffuse occurs throughout the body. It usually
affects the skin as well as other body parts, such as the lungs, kidneys,
heart, bowels, blood vessels and joints. Depending on the areas affected,
this type can cause problems such as high blood pressure, muscle weakness,
trouble swallowing, or shortness of breath.
Diffuse scleroderma may progress slowly in some people
and more rapidly in others. However, with proper management, it can be
controlled.
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