Dr. M.J. Bazos, MD.
Scleroderma is a multisystem connective tissue
disease in which the skin and a variety of internal organs progressively
degenerate, thicken, and lose flexibility. It is characterized by widespread
inflammation, stiffness, and loss of function of the connective tissue (the
framework for all body tissues). It affects multiple organs and tissues,
including the skin, blood vessels, joints, heart, kidneys, lungs, esophagus (the
food tube connecting the throat to the stomach), fingers, and toes. The exact
cause is unknown. Scleroderma is an autoimmune disorder (i.e., a condition in
which your immune system mistakenly attacks normal parts of the body, resulting
in tissue injury and disease). Scleroderma is uncommon. It affects women four
times more frequently than men. It typically begins in adults aged 30–50
years. The transmission of this condition is unknown, although it may be
genetic. Individuals with scleroderma tend to have poor circulation. As a
result, exposure to extremely cold weather and air conditioning may aggravate
your scleroderma. Diagnosis is usually based upon the inflammation,
degeneration, thickening and stiffness of your skin and other internal organs.
Your doctor may order blood tests and perform a skin biopsy (removal of a small
piece of skin or other tissue) for laboratory evaluation to assist in diagnosis.
No cure is known. Scleroderma is a chronic condition in which the skin and
multiple internal organs slowly and progressively degenerate, lose function, and
become thick and stiff. Treatment aims to control symptoms and to lessen the
severity of inflammation, but does not cure
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Individuals with scleroderma
frequently have multiple symptoms because of the widespread nature of the
disease. The skin typically loses flexibility and becomes coarse and thickened.
These changes produce a masklike appearance of the face, thinning of the lips,
and furrowing around the mouth. The fingers become thickened and stiff,
resulting in pain as well as loss of mobility. Many individuals have pain,
swelling, and stiffness of the joints, muscle aches, weakness, and fatigue.
Other symptoms include heartburn, difficulty swallowing (especially with solid
foods), constipation or diarrhea, abdominal bloating, and weight loss. With lung
involvement, symptoms include shortness of breath with mild activity, and a dry
cough. Some individuals with scleroderma have the CREST
calcification of the skin, especially the fingers and hands, associated with
drainage of white paste from
phenomenon: a disorder of the blood vessels resulting from inflammation,
thickening, and loss of flexibility. It typically affects the
arteries of the hands and feet.
Symptoms include discoloration of the fingertips and toes (pale, blue/purple, or
red) with exposure to cold temperatures. Other symptoms include pain, tingling,
and coldness of the fingers and toes.
• Esophageal dysfunction:
heartburn, difficulty swallowing (especially solid foods), and gastroesophageal
reflux (acid from the stomach irritates the lining of the
thickening and stiffness of the skin of the fingers and toes, associated with a
waxy and shiny appearance.
Telangiectasia: a cluster of dilated, small blood vessels of the skin.
Because scleroderma is so widespread, it can cause multiple complications. The
most common cause of death is kidney failure. Other complications include heart
failure, abnormal heart rhythms, high blood pressure, poor lung function, poor
wound healing, gangrene, bleeding, and
is incurable, and its course is characterized by a gradual and relentless
progression of skin and internal organ damage. Treatment aims to control
symptoms, lessen the severity of your illness, and to treat/prevent
complications. Your treatment will depend on the type and severity of your
symptoms. Treatment consists of general measures and medications. General
measures to follow include:
smoking, because smoking can worsen the function of your heart, lungs, and blood
vessels, as well as irritate the lining of your digestive
2. If you have heartburn or
gastroesophageal reflux, several measures may reduce the severity of your
symptoms. Eliminating or reducing cigarette smoking, as well as the intake of
alcohol and caffeine, can decrease reflux and heartburn. Also, avoid eating
within 2 hours of bedtime or lying down. When lying down, raise the head of your
bed by 6–8 inches and sleep on two or three
3. Avoid exposure to extremely
cold temperatures, and be careful of air conditioning, which may worsen poor
circulation. Wear warm, protective clothing, especially gloves and
4. Avoid skin injuries and burns.
Adjusting the hot water heater in your home to limit the water temperature to a
maximum of 120°F reduces your risk of sustaining burns. Also, check the
water temperature with a body part that has good circulation before exposing
fingers and toes with poor circulation.
Apply heat to improve joint pain and
6. Individuals with scleroderma
frequently become depressed or experience other psychological conditions. If you
feel depressed or are having difficulty coping with your illness, talk to your
doctor about the best treatment options, including psychological counseling.
Biofeedback techniques are also effective in increasing circulation in some
individuals. The following medications are helpful in treating
1. Corticosteroids can relieve
inflammatory symptoms. Side effects are more likely with higher doses and
include increased susceptibility to infection, swelling, diabetes mellitus,
osteoporosis (thinning of your bones), and injury to the lining of the digestive
system. Do not stop steroid medication without first consulting your doctor
because abrupt cessation can result in severe weakness, fatigue, and low blood
2. Skin lotions, moisturizers,
and bath oils are useful in softening the
3. Over-the-counter antacids or
prescription medications are useful in treating gastroesophageal
4. Your doctor may prescribe other
medications to treat complications of other organ systems. Examples are blood
pressure medicines, antibiotics for infections, and aspirin or anti-inflammatory
medications for arthritis.
• Take medications as
prescribed by your doctor.
your doctor of all other medications, including over-the-counter medications,
that you are taking. Continue these medications unless your doctor instructs you
to stop them.
• Read the labels of
medicines and follow all instructions. Consult your doctor if you have any
concerns or if you have possible side effects caused by the
• When lying down, raise
the head of your bed by 6–8 inches and sleep on two or three
• Regular exercise improves
blood pressure and circulation and maintains heart and lung function. Exercise
also helps keep the skin and joints flexible and maintains good muscle tone.
Exercise regularly as your strength
• Stop smoking, because
smoking can worsen the function of your heart, lungs, and blood vessels, as well
as irritate the lining of your digestive
• Do not stop your
medicine or change the prescribed dose without consulting your
• Do not exceed recommended
doses of medicines, because higher doses can increase your risk of toxic
• Do not abruptly stop
corticosteroids because you may experience a rebound worsening of your
condition. Abrupt cessation of these medicines can result in severe weakness,
fatigue, and low blood pressure. Consult your doctor before stopping these
• Avoid skin injury,
burns, and cuts to reduce your risk of skin ulceration, infections, and
• If you have
gastroesophageal reflux symptoms or heartburn, eliminate cigarette smoking, as
well as your intake of alcohol and caffeine, to reduce the irritation of the
lining of the esophagus and to decrease your symptoms. Avoid eating within 2
hours of bedtime to reduce symptoms. Avoiding spicy foods can also improve
When To Call Your
• If you have any signs or
symptoms of infection, including redness around a wound or skin lesion, purulent
discharge (pus) from a wound, increased pain or swelling of wounds or lymph
nodes, and fever.
• If you notice
that signs or symptoms of your condition are becoming worse, or if new signs or
• If you have signs
or symptoms of systemic illness, including fever, lethargy, confusion, or
• If you have
new or unexplained symptoms, which may indicate a complication of your condition
or side effects from medications.