Scleroderma

The following is information on Scleroderma, one of the 15 health conditions covered by the VA for Camp Lejeune victims. Information has been compiled from sources such as Scleroderma Foundation, Mayo Clinic and others.

What is Scleroderma?

Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases.

Symptoms

These vary, depending on which parts of your body are involved:

• Skin. Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it’s so tight, and movement of the affected area may be restricted.
• Fingers or toes. One of the earliest signs of scleroderma is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, pain or color changes in the fingers or toes. Called Raynaud’s phenomenon, this condition also occurs in people who don’t have scleroderma.
• Digestive system. In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients if their intestinal muscles aren’t moving food properly through the intestines.
• Heart, lungs or kidneys. Rarely, scleroderma can affect the function of the heart, lungs or kidneys. These problems can become life-threatening.

Causes

For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen.

Complications

Scleroderma complications range from mild to severe and can affect your:

• Fingertips. The variety of Raynaud’s phenomenon that occurs with scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores (ulcers). In some cases, gangrene and amputation may follow.
• Lungs. Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).
• Kidneys. When scleroderma affects your kidneys, you can develop an elevated blood pressure and an increased level of protein in your urine. More-serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
• Heart. Scarring of heart tissue increases your risk of abnormal heartbeats (arrhythmias) and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart (pericarditis). Scleroderma also can raise the pressure on the right side of your heart and cause it to wear out.
• Teeth. Severe tightening of facial skin can cause your mouth to become smaller and narrower, which may make it hard to brush your teeth or to even have them professionally cleaned. People who have scleroderma often don’t produce normal amounts of saliva, so the risk of dental decay increases even more.
• Digestive system. Digestive problems associated with scleroderma can lead to acid reflux and difficulty swallowing — some describe feeling as if food gets stuck midway down the esophagus — as well as bouts of constipation alternating with episodes of diarrhea.
• Sexual function. Men who have scleroderma often experience erectile dysfunction. Scleroderma may also affect the sexual function of women, by decreasing sexual lubrication and constricting the vaginal opening.

How Is Scleroderma Diagnosed?

Doctors diagnose scleroderma using:

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

Scleroderma can be hard to diagnose. Other diseases can have similar symptoms. It is easier to diagnose if you have:

• Common symptoms
• Skin that gets thick fast.

Treatment

A rheumatologist (a doctor who treats arthritis and other diseases that cause swelling in the joints) may lead your health care team and refer you to other health experts for problems with:

• Skin
• Kidneys
• Heart
• Digestion
• Lungs
• Teeth
• Movement
• Speech.

Scleroderma has no cure. But symptoms and damage can be reduced. 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. It can affect the fingers, feet, and hands. It makes them change color if you are too cold or anxious. To help, you can:

• Not smoke.
• Dress warm, and keep hands and feet warm.
• Do exercises that relax the body.
• Ask about medicines that open small blood vessels and help with blood flow.
• Ask about medicines that treat skin sores and ulcers.

Stiff, Painful Joints

Stiffness and pain come from hard skin around joints and joint swelling. To help, you can:

• Do stretching exercises that help with joint motion.
• Exercise regularly (swimming is best).
• Take medicine to help ease pain or swelling. Ask your doctor which are the best for you to take.
• Learn to do daily tasks in ways that put less stress on the joints.

Skin Problems

With scleroderma, collagen builds up in the skin. Too much of it can make your skin dry and stiff. To help, you can:

• Use oil-based creams and lotions after every bath.
• Use sunscreen.
• Use a humidifier at home.
• Avoid hot baths or showers.
• Avoid strong soaps, cleaners, and chemicals. Wear rubber gloves if you have to use those products.
• Exercise regularly.

Dry Mouth and Dental Problems

If you have tight skin on your face, you may have trouble caring for your teeth. Dry mouth speeds up tooth decay. Harm to tissues in the mouth can loosen teeth. To avoid 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.
• Ask your dentist about special rinses and toothpastes.
• Learn ways to keep your mouth and face flexible.
• Keep your mouth moist. You can drink lots of water or suck on ice chips. You can also chew gum or suck on hard candy that has no sugar added.
• 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 blocks 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.
• Ask your doctor about medicines for diarrhea, constipation, and heartburn.

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.

As soon as your skin starts to thicken, see your doctor. Get regular flu and pneumonia shots.

Heart Problems

Problems include:

• Scarring and weakness
• Swelling of the heart muscle
• A heartbeat that isn’t normal.

These problems can all be treated.

Kidney Problems

Scleroderma can cause very high blood pressure and kidney failure in some people. Learn to spot problems right away.

You should:

• Check your blood pressure often.
• Check your blood pressure 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 include:

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

Additional Information & Links:

Mayo Clinic: http://www.mayoclinic.org/diseases-conditions/scleroderma/basics/definition/con-20021378

Scleroderma.org – http://www.scleroderma.org/site/PageServer?pagename=body_tool#.V5EpiKJirBW

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Information Clearinghouse – National Institutes of Health

1 AMS Circle
Bethesda, MD 20892-3675
Phone: 301-495-4484
Toll Free: 877-22-NIAMS (877-226-4267)
TTY: 301-565-2966
Fax: 301-718-6366
Email: NIAMSinfo@mail.nih.gov
Website: http://www.niams.nih.gov