Dr. M.J. Bazos, MD. Patient Handout

About Your Diagnosis

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 scleroderma.

Living With Your Diagnosis
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 syndrome:
Calcinosis: calcification of the skin, especially the fingers and hands, associated with drainage of white paste from ulcerations.
Raynaud’s phenomenon: a disorder of the blood vessels resulting from inflammation, thickening, and loss of flexibility. It typically affects the small
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 esophagus).
Sclerodactyly: 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 bruising.

Scleroderma 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:
1. Stop smoking, because smoking can worsen the function of your heart, lungs, and blood vessels, as well as irritate the lining of your digestive system.
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 pillows.
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 socks.
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.
5. Apply heat to improve joint pain and stiffness.
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 scleroderma:
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 pressure.
2. Skin lotions, moisturizers, and bath oils are useful in softening the skin.
3. Over-the-counter antacids or prescription medications are useful in treating gastroesophageal reflux.
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.

The DOs
• Take medications as prescribed by your doctor.
• Inform 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 medications.
• When lying down, raise the head of your bed by 6–8 inches and sleep on two or three pillows.
• 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 permits.
• Stop smoking, because smoking can worsen the function of your heart, lungs, and blood vessels, as well as irritate the lining of your digestive system.

The DON’Ts
• Do not stop your medicine or change the prescribed dose without consulting your doctor.
• Do not exceed recommended doses of medicines, because higher doses can increase your risk of toxic effects.
• 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 medicines.
• Avoid skin injury, burns, and cuts to reduce your risk of skin ulceration, infections, and gangrene.
• 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 symptoms.

When To Call Your Doctor
• 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 symptoms develop.
• If you have signs or symptoms of systemic illness, including fever, lethargy, confusion, or respiratory distress.
• If you have new or unexplained symptoms, which may indicate a complication of your condition or side effects from medications.