CREST Syndrome (Limited Scleroderma)
Systemic scleroderma, or systemic sclerosis, is the term for the disease that is characterized by diffuse thickening of the skin, but may also involves the tissues beneath to the blood vessels and major organs. Systemic sclerosis is typically categorized into diffuse and limited disease.
Limited scleroderma disease develops gradually and generally affects the skin in the fingers, hands, face, lower arms and legs. Other symptoms may develop before or after the onset of skin thickening and when existing together are classified as CREST syndrome.
The following are the signs or symptoms comprising the CREST syndrome (acronym):
- Calcinosis (KAL-sin-OH-sis): The formation of calcium deposits in the connective tissues, which can be detected by x ray. They are typically found on the fingers, hands, face, and trunk and on the skin above elbows and knees. When the deposits break through the skin, painful ulcers can result.
- Raynaud’s (ray-NOHZ) phenomenon: A condition in which the small blood vessels of the hands and/or feet contract in response to cold or anxiety. As the vessels contract, the fingers or toes turn white and cold, then blue. As blood flow returns the digits become red and warm. With severe disease, the fingertip tissue may suffer damage, leading to ulcers, scars, or gangrene. It is often the first symptoms and will occur prior to skin changes.
- Esophageal (eh-SOFF-uh-GEE-ul) dysfunction: Impaired muscle contracting function of the esophagus (the tube connecting the throat and the stomach). This can result in swallowing difficulties and chronic heartburn or inflammation in the esophagus.
- Sclerodactyly (SKLER-oh-DAK-till-ee): Thick and tight skin on the fingers, resulting from deposits of excess collagen within skin layers. The condition makes it harder to bend or straighten the fingers. The skin may also appear shiny and darkened, with hair loss.
- Telangiectasias (tel-AN-jee-ek-TAY-zee-uhs): Small red spots on the hands, face and chest that are caused by the swelling of tiny blood vessels. While not painful, these red spots can create cosmetic problems.
Because scleroderma (limited or diffuse) may affect different organ systems, one may have several different doctors involved in their care. The care will typically be managed by a rheumatologist, a specialist who treats diseases of the joints, bones, muscles, and immune system. Your rheumatologist may refer you to other specialists, depending on the specific disease manifestation, including a dermatologist for the treatment of skin, a gastroenterologist for problems of the digestive tract, and a pulmonary specialist for lung involvement.
Currently, there is no treatment that controls or stops the underlying autoimmune procees; the overproduction of collagen Thus, treatment and management es focused on relieving symptoms and limiting damage. Your treatment will depend on the particular disease complications. This may include medications provided by your doctors or the use of lifestyle modifications to decrease symptoms.
The following are potential signs or symptoms that may arise during the course of systemic scleroderma. There are recommendations both medical and other to treat them.
[Note: This is not a complete listing of problems or their treatments. Different people experience different problems with scleroderma and not all treatments work equally well for all people. Work with your doctor to find the best treatment for your specific symptoms.]
- One of the most common scleroderma-related symptoms. Raynaud’s phenomenon can be uncomfortable and can lead to painful skin ulcers on the fingertips. Smoking makes the condition worse. The following measures are recommended to prevent its complications:
- Avoid smoking! Tobacco smoking narrows the blood vessels and may worsen Raynaud’s phenomenon.
- Dress warmly, with special attention to hands and feet. Dress in layers, wear gloves and try to stay indoors during cold weather.
- Use biofeedback (to control various body processes that are not normally thought of as being under conscious control) and relaxation exercises.
- For severe cases, speak to your doctor about medications to help open the small blood vessels in the hands and improve circulation. Other drugs are in development and may become available in the future.
- If Raynaud’s leads to skin sores or ulcers, antibiotic ointments will decrease your risk for infections. You may need to adjust your medications used to open the small blood vessels, discuss this with your doctor. Severe ulcerations on the fingertips can be treated with bioengineered skin.
Stiff, painful joints:
- In diffuse systemic sclerosis, hand joints can stiffen because of hardened skin around the joints or inflammation of the joints themselves. The following may help:
- Exercise regularly. Ask your doctor or physical therapist about an exercise program that will increase and maintain range of motion in affected joints. Swimming can help maintain muscle strength, flexibility, and joint mobility.
- Acetaminophen or other over-the-counter or prescription nonsteroidal anti-inflammatory drug, as recommended by your doctor, may help relieve joint or muscle pain. Prescription-strength drugs may be necessarily to ease severe pain and inflammation.
- Learn to do things in a new way. A physical or occupational therapist can help to modify the way you perform daily tasks, such as lifting and carrying objects or opening doors, in ways that puts less stress on painful and stiff joints.
- When too much collagen builds up in the skin, it crowds out sweat and oil glands, causing the skin to become dry and stiff. If your skin is affected, you may need to see a dermatologist. To ease dry skin, try the following:
- Apply oil-based creams and ointments frequently, and always after bathing.
- Apply sunscreen (SPF 50) with expected sun exposure.
- Use humidifiers to moisten the air in your home in colder winter climates. (Clean humidifiers often to stop bacteria from growing in the water.)
- Avoid very hot baths and showers, as hot water dries the skin.
- Avoid harsh soaps, household cleaners, and caustic chemicals. If not possible, be sure to wear rubber gloves when using such products.
- Exercise regularly. Exercise, especially swimming, stimulates blood circulation to affected areas.
Dry mouth and dental problems:
- Dental problems are common in people with scleroderma for a number of reasons. Tightening of the facial skin can make the mouth opening smaller and narrower, making dental care difficult. Dry mouth due to salivary gland damage speeds up tooth decay. Damage to connective tissues in the mouth can lead to loose teeth. You can avoid tooth and gum problems in several ways:
- Brush and floss your teeth regularly. (If hand pain and stiffness make this difficult, consult your doctor or an occupational therapist about specialty toothbrush handles and devices.)
- Regular dental checkups. Contact your dentist immediately if you experience mouth sores, mouth pain, or loose teeth.
- If decay is a problem, ask your dentist about fluoride rinses or prescription toothpastes that remineralize and harden tooth enamel.
- Consult a physical therapist about facial exercises to maintain mouth and face flexibility.
- Keep your mouth moist by drinking plenty of water, sucking ice chips, using sugarless gum and hard candy, and avoiding mouthwashes with alcohol. If dry mouth still bothers you, ask your doctor about a saliva substitute or a prescription medication to stimulate the flow of saliva.
Gastrointestinal (GI) problems:
- Systemic sclerosis can affect any part of the digestive system. As a result, you may experience heartburn, difficulty swallowing, early satiety (the feeling of being full after you’ve barely started eating), or diarrhea, constipation, and gas. In cases where the intestines are damaged, your body may have difficulty absorbing nutrients from food. Although GI problems vary, here are some things that might help at least some of the problems you have:
- Eat small, frequent meals.
- Raise the head of your bed with blocks, and stand or sit upright for at least an hour (preferably two or three) after eating to keep stomach contents from backing up into the esophagus.
- Avoid late-night meals, spicy or fatty foods, and alcohol and caffeine, which can aggravate GI discomfort.
- Chew foods well and eat moist, soft foods. If you have difficulty swallowing or if your body doesn’t absorb nutrients properly, your doctor may prescribe a special diet.
- Ask your doctor about prescription medications for problems such as diarrhea, constipation, and heartburn. Drugs called proton pump inhibitors are highly effective for heartburn. Oral antibiotics may stop bacterial overgrowth in the bowel that can be a cause of diarrhea in some people with systemic sclerosis.
- About 10 to 15 percent of people with systemic sclerosis develop severe lung disease, which comes in two forms: pulmonary fibrosis (hardening or scarring of lung tissue because of excess collagen) and pulmonary hypertension (high blood pressure in the artery that carries blood from the heart to the lungs). Treatment for the two conditions is different.
- Pulmonary fibrosis may be treated with drugs that suppress the immune system such as cyclophosphamide (Cytoxan) or mycophenolate (Cellcept), along with low doses of corticosteroids. There is a new class of anti-fibrotic medications available, inquire to your doctor whether these are right for you.
- Pulmonary hypertension may be treated with drugs that dilate the blood vessels of the lungs.
- Regardless of the problem or its treatment, your role in the treatment process is essentially the same, minimizing lung complications. It is imperative to work closely with your medical team and;
- Watch for signs of lung disease, including fatigue, shortness of breath or difficulty breathing, and swollen feet. Report these symptoms to your doctor.
- Have your lungs closely monitored, using standard lung-function tests. During the early stages of skin thickening these tests, can identify problems at the earliest and most treatable stages.
- Get regular flu and pneumonia vaccines as recommended by your doctor. Contracting either illness could be dangerous for a person with lung disease.