Scleroderma
Medical Author: William C. Shiel Jr., MD, FACP, FACR
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Scleroderma Elbow Tip Pains
Medical Author: William C. Shiel Jr., MD, FACP, FACR
 Scleroderma is a chronic condition that causes inflammation and
thickening of the skin. The cause of scleroderma is not known. Researchers have
found some evidence that genes are important factors, but the environment seems
to also play a role. This means that inheritance at least play a partial role.
It is not unusual to find other autoimmune diseases in families of scleroderma
patients. Some evidence for the role genes may play in leading to the
development of scleroderma comes from the study of Choctaw Native Americans who
are the group with the highest reported prevalence of the disease. The disease
is more frequent in females than in males.
A common area of involvement of this condition is
the skin over the tips of the elbows (the olecranon area). When this skin
is involved, it can lead to troublesome irritation of the tips of the elbows
with tenderness noted when any pressure is applied.
Recently, I saw a patient in a follow up visit who is a veterinarian with
scleroderma with this particular complication. We had been treating the
condition with a variety of topical lubricants and emollients, but to no avail.
She returned at this visit to tell me of a surprisingly successful treatment
that she had found on her own.
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What is scleroderma?
Scleroderma is an autoimmune disease of the
connective tissue. Autoimmune diseases are illnesses which occur when the body's tissues are attacked by its own immune system. Scleroderma is characterized by
the formation of scar tissue (fibrosis) in the skin and organs of the
body. This leads to thickness and firmness of involved areas. Scleroderma, when it's diffuse or widespread over the body, is also referred to as systemic sclerosis.
The cause of scleroderma is not known. Researchers
have found some evidence that genes are important factors, but the environment seems to also play a role. The result is activation of the immune system, causing injury to tissues that result in injury similar to scar tissue formation. The fact that genes seem to cause a predisposition to developing scleroderma means that inheritance at least plays a partial role. It is not
unusual to find other autoimmune diseases in families of scleroderma
patients. Some evidence for the role genes may play in leading to the development of scleroderma comes from the study of Choctaw Native Americans who are the group with the highest reported prevalence of the disease. The disease is more frequent in females than in males.
How is scleroderma classified?
Scleroderma can be classified in terms of the degree and location of the
skin involvement. Accordingly, scleroderma has been categorized into two
major groups, diffuse and limited.
The diffuse form of scleroderma (systemic sclerosis) involves symmetric thickening of skin
of the extremities, face, and trunk (chest, back, abdomen, or flanks) which
can rapidly progress to hardening after an early inflammatory phase. Organ
disease can occur early on and be serious. Organs affected include the
esophagus, bowels, lungs with scarring (fibrosis), heart, and kidneys.
High blood pressure can be a troublesome side effect.
The limited form of scleroderma tends to be confined to the skin of the
fingers and face. The skin changes and other features of disease tend to
occur more slowly than in the diffuse form. Because a characteristic
clinical pattern can occur in patients with the limited form of
scleroderma, this form has taken another name which is composed of the
first initials of the common components. Thus, this form is also called
the CREST variant of scleroderma. This name represents the following
features:
C...Calcinosis refers to the formation of tiny deposits of calcium in
the skin. This is seen as hard whitish areas in the superficial skin,
commonly overlying the elbows, knees, or fingers. These firm deposits can
be tender, can become infected, and can fall off spontaneously or require
surgical removal. This is the least common of the CREST scleroderma variant features.
R...Raynaud's phenomenon
refers to the spasm of the tiny artery vessels supplying blood to the
fingers, toes, nose, tongue, or ears. These areas turns blue, white, then
red after exposure to extremes of cold, or even sometimes with extremes of
heat or emotional upset.
E...Esophagus disease in scleroderma is characterized by poorly
functioning muscle of the lower two-thirds of the esophagus. This can lead to an
abnormally wide esophagus which allows stomach acid to backflow into the
esophagus to cause heartburn, inflammation, and potentially scarring. This
can eventually lead to difficulty in passing food from the mouth through
the esophagus into the stomach. Symptoms of heartburn are treated
aggressively in patients with scleroderma in order to prevent injury to
the esophagus.
S...Sclerodactyly refers to the localized thickening and tightness of
the skin of the fingers or toes. This can give them a "shiny" and slightly puffy
appearance. The tightness can cause severe limitation of motion of the
fingers and toes. These skin changes generally progress much slower that
those of patients with the diffuse form of scleroderma.
T...Telangiectasias are tiny red areas, frequently on the face, hands
and in the mouth behind the lips. These areas blanch when they are pressed
upon and represent dilated capillaries.
Patients can have variations of CREST, for example, CRST, REST, ST, etc.
Patients can also have "overlap" illness with features of both
CREST and the diffuse form of scleroderma. Some patients have overlaps of
scleroderma and other connective tissue diseases, such as rheumatoid
arthritis, systemic lupus erythematosus, and polymyositis. When features of scleroderma
are present along with features of polymyositis and systemic lupus erythematosus, the
condition is referred to as mixed connective tissue disease (MCTD).
Finally, scleroderma skin changes can be very localized. Morphea is scleroderma
skin that is localized to a patchy area of the skin that becomes hardened and slightly
pigmented. Sometimes morphea can cause multiple lesions in the skin. Morphea is not associated with disease elsewhere in the body. Linear scleroderma
is scleroderma that is localized usually to a lower extremity, frequently presenting as
a strip of hardening skin down the leg of a child. Linear scleroderma in children can
stunt bone growth of the affected limb. Sometimes linear scleroderma is associated with a "satellite" area of a patch of localized scleroderma skin, such as on the abdomen.
Next: What are symptoms of scleroderma? »
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