Scleroderma

Scleroderma is a group of diseases that cause abnormal growth, hardening, and thickening of connective tissue. There are two major classifications of scleroderma, localized scleroderma and systemic sclerosis.

The symptoms of scleroderma depend on the part of the body affected and the extent which they are affected. Localized Scleroderma may produce waxy patches on the skin of varying sizes, shapes and color. The skin under the patches may thicken. Systemic scleroderma can involve the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart and other internal organs. Symptoms may include calcium deposits in connective tissues, raynaud's phenomenon, swelling of the esophagus, and thick and tight skin on the fingers.

The cause of scleroderma is unknown. Although scleroderma can develop at every age group, it most often appears between the ages of 25 to 55. It is generally believed that the disease develops in response to inflammation and the overproduction of collagen.

There is no cure for scleroderma, but there are treatments for symptoms and complications. Calcium Channel blockers can be used to treat Raynaud’s phenomenon and treat high blood pressure. Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin) and naproxen (Naprosyn, Aleve), are used to help manage inflammation, swelling, stiffness and joint pain. Corticosteroids are used to reduce swelling, redness, itching and allergic reactions. 

The symptoms of scleroderma can vary for each person, and the effects of scleroderma can range from very mild to life threatening. The severity will depend on the part of the body affected and the extent which they are affected.

There are two major classifications of scleroderma: localized scleroderma and systemic sclerosis.

Localized Scleroderma. The changes are usually found in a few places on the skin or muscles, and rarely spread elsewhere.

Someone with localized scleroderma can notice waxy patches on the skin of varying sizes, shapes and color. The skin under the patches may thicken.

Or, someone may notice a streak or line of hardened, waxy skin on an arm or leg or on the forehead. Sometimes it forms a long crease on the head or neck. If this is the case, motion of the joints may be affected.

Systemic scleroderma. Systemic scleroderma can involve the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart and other internal organs. It can also affect blood vessels, muscles and joints.

• People with systemic scleroderma can either have diffuse or limited disease.
  • In diffuse scleroderma, skin thickening occurs faster and involves more skin areas. 
  • Limited scleroderma involves a slower and more benign illness.

Symptoms of scleroderma include:

• Calcium deposits in connective tissues
• Raynaud's phenomenon, which is a narrowing of blood vessels in the hands or feet
• Swelling of the esophagus
• Thick and tight skin on your fingers
• Red spots on your hands and face

The cause is unknown. Scleroderma is not contagious, inherited, infectious, cancerous or malignant.

Although scleroderma can develop at every age group, it most often appears between the ages of 25 to 55.

Risk factors:

• Scleroderma is more common in women than men.
• Localized scleroderma is more common in children.
• Systemic scleroderma is more common in adults.
• Race and ethnic background
  • Localized types of scleroderma tends to be more common in people of European descent compared to African Americans.
  • Systemic types of scleroderma are more common in African Americans in comparison to those European descent.

Doctors diagnose scleroderma using:

• Your medical history
• A physical exam
• Lab tests
• A skin biopsy

Below are some problems and treatments for systemic scleroderma. These problems don’t happen with localized scleroderma.

Raynaud’s Phenomenon

Most people with scleroderma have Raynaud’s phenomenon. Raynaud’s phenomenon is when your fingers, feet, and hands change color, if you are too cold or anxious. To help, you can:

• Avoid smoking
• Dress warm, and keep hands and feet warm
• Do exercises that relax the body

Stiff, Painful Joints

Hard skin around joints and joint swelling can cause stiffness and pain. To help, you can:

• Stretch to promote joint motion
• Exercise regularly
• Avoid putting too much stress on the joints

Skin Problems

With scleroderma, collagen builds up in the skin. As a result, your skin may appear dry and stiff. To help, you can:

• Use oil-based creams and lotions after taking a bath
• Use sunscreen
• Use a humidifier at home
• Avoid hot baths or showers
• Avoid strong soaps, cleaners, and chemicals
• Exercise

Dry Mouth and Dental Problems

• Brush and floss your teeth each day
• Have frequent dental checkups
• See your dentist if you have mouth sores, mouth pain, or loose teeth
• Learn ways to keep your mouth and face flexible
• Keep your mouth moist
• Avoid mouthwash that has alcohol

If dry mouth still bothers you, ask your doctor about helpful medicines.

Gastrointestinal Problems

Digestive problems can include:

• Heartburn
• Trouble swallowing
• Feeling full as soon as you start eat eating
• Diarrhea, constipation, and gas

To help, you can:

• Eat small, frequent meals
• Stand or sit for 1 to 3 hours after eating
• Use wedges to raise the head of your bed
• Avoid late-night meals, spicy or fatty foods, alcohol, and caffeine
• Eat moist, soft foods, and chew them well

Lung Damage

Problems include:

• Some loss of lung function
• Severe lung disease
• Scarring of lung tissue
• High blood pressure in the artery that carries blood from the heart to the lungs

Watch for signs of lung disease, such as:

• Fatigue
• Shortness of breath
• Problems with breathing
• Swollen feet

Heart Problems

Problems include:

• Scarring and weakness
• Swelling of the heart muscle
• An abnormal heartbeat

Kidney Problems

Scleroderma can cause very high blood pressure and kidney failure in some people.

You should:

• Check your blood pressure often and if you have new symptoms
• Call your doctor if your blood pressure is higher than normal
• Take the medicines your doctor prescribes

Cosmetic Problems

Scleroderma can damage your skin and change how it looks. These skin changes can affect your self-image. Ways to fix skin damage may include:

• Lasers that take away red spots on the hands and face
• Plastic surgery in areas where the disease is not active

Get regular flu and pneumonia shots.

Scleroderma has no cure, but various treatments can control symptoms and complications.

Since there is so much variation from one person to another, treatments will vary greatly.

Below are some problems and treatments for systemic scleroderma. These problems don’t happen with localized scleroderma.

Raynaud’s Phenomenon

Calcium Channel blockers, such as nifedipine (Procardia), amlodipine (Norvasc), and verapamil (Calan, Verelan), can be used to treat Raynaud’s phenomenon and treat high blood pressure.

Angiotensin II Receptor Antagonists , such as losartan (Cozaar) and valsartan (Diovan)  as well as Angiotensin Converting Enzyme (ACE) inhibitors, such as captopril (Capoten), benazepril (Lotensin), lisinopril (Prinivil, Zestril) can also be used.

Joint Stiffness and Pain

Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin) and naproxen (Naprosyn, Aleve), are used to help manage inflammation, swelling, stiffness and joint pain.

COX-2 inhibitors such as celecoxib (Celebrex) can be used to relieve symptoms such as inflammation, swelling, stiffness and joint pain. Celecoxib (Celebrex) is a type of NSAID.

Analgesics such as acetaminophen (Tylenol) is used to relieve pain (headaches, muscle aches) and reduce fever.

Low-Dose Corticosteroids, such as prednisone, are used to give relief for inflamed areas of the body by reducing swelling, redness, itching and allergic reactions.

Narcotic analgesics, such as acetaminophen/hydrocodone (Vicodin), act  to relieve pain.

Gastrointestinal Reflux

Antacids, such as Maalox and Mylanta can be given to relieve heartburn, sour stomach or acid indigestion.

Proton Pump Inhibitors, such as omeprazole (Prilosec) and esomeprazole (Nexium), reduce stomach acid production, minimize gastrointestinal reflux disease, and help prevent esophageal ulcers.

Your doctor may prescribe other medications based on your symptoms.

Current studies include:

• Research looking at how genes may be involved in scleroderma
• Medications to treat/prevent skin from getting thick
• Medications to treat kidney and lung problems
• Medications to treat scleroderma and its various organ complications
• Methods to identify and diagnose scleroderma much earlier

Research and new treatments are helping people with scleroderma feel better and stay active much longer than they did in the past.