Answers to Some Frequently Asked Questions

Originally published in Scleroderma Voice, 2004 #2

The Scleroderma Foundation receives questions on a daily basis from patients seeking answers to health issues.

Although scleroderma is a highly individualized disease, we have found that some concerns are very common among those affected. We thought this forum would be a perfect opportunity to revisit some of these concerns and address them in detail. The focus this month will be issues related to skin involvement in scleroderma.

We hope you find this section informative. However, we understand individual circumstances are unique, and ask that you seek the guidance of your healthcare professional to obtain the treatment plan best suited to your specific health history.

Calcinosis

Calcinosis is a condition that sometimes occurs in both limited (CREST) and diffuse cutaneous systemic sclerosis. It is characterized by calcium deposits in the soft tissue (just below the skin's surface) of the fingers, hands, elbows, feet, knees, or even buttocks. Diagnosis can be easily confirmed by x-ray.

When calcium deposits occur in the hand or fingers, they can impair function due to their painful nature. It is important to protect the area in which they occur. Padding the area with bandages can be most helpful to avoid the inevitable bumping or banging of the area during your daily activities.

Many patients have become quite creative devising techniques to cushion these areas. Some have found the foot-care section of their local drug stores to be a source of helpful products. Toecaps and tubular cushioning bandages, commonly used for corn protection, can be a wonderful way to protect the painful sites on your fingers.

Minimizing Calcinosis Discomfort

Unfortunately, there is no effective way to prevent calcium deposits, but there are some therapies to minimize the discomfort associated with their presence. Warm-water soaks can be helpful in decreasing the inflammation and pain. Cushioning protection as mentioned above, and good skin care, can help to minimize breakdown of the skin over an affected area.

In an effort to decrease inflammation associated with calcinosis, some physicians may prescribe the medications colchicine, low-dose warfarin, or minocycline.

It is important to note that there is no one medication proven to be universally effective for the treatment of calcinosis. Occasionally antibiotics may be prescribed to treat a localized infection that may occur in the area of the deposit. Keep in mind that the area may be affected by inflammation of the tissue surrounding the calcium deposit and may become reddened as a result. It can be difficult to determine if this is actually just inflammation or a sign of infection.

Preventing Calcinosis Infection

Even patients who are meticulous in the care of their hands may experience a calcium deposit that opens up at the surface of the skin, producing a milky white material.
Extreme care should be taken to minimize infection over this area. Soap and water cleansing, as well as sterile bandaging to protect the open area from infection are very important.

If you develop signs of infection in the open area such as redness, swelling, excessive pain, drainage, or fever, please contact your healthcare provider immediately for treatment. In this instance antibiotic therapy would be necessary.

In severe cases surgical removal of the deposit may be necessary, but recurrence is common.

Never, ever, pick, dig, or probe at a calcium deposit, no matter how great the urge to do so, because this can lead to infection and other serious complications. Always consult your physician if an area is painful or swollen or shows any of the other signs of infection outlined above.

The Sun and Your Skin

Many persons with localized or systemic scleroderma enjoy spending time in the sun for family activities, or feel that it has a therapeutic effect on their skin. Anyone who spends time in the sun should take precautions to protect themselves from skin cancer.

A few things to keep in mind:

1. ALWAYS wear sunscreen or sunblock when exposing your skin to the suns rays. Those of you who like to tan should remember that you can still get a tan while wearing sunscreen. If you have sensitive skin, you can buy sunscreen products especially developed with you in mind. If you are swimming or sweating, remember to reapply sunscreen occasionally. You may also want to consider using a product labeled waterproof or "sport." Wearing a broad-brimmed hat can give you extra protection from the sun's harmful rays.
2. The sun is drying to the skin. Remember to moisturize your skin frequently with a product that contains lanolin to minimize this effect. Dry skin may increase itchy skin!
3. Hypopigmented (decreased color) areas of your skin as a result of your scleroderma (this is most common in people of color) are at greater risk of sunburn and skin cancers. Hyperpigmented (darkened) areas may become more pronounced with sun exposure.

Skin Ulcerations

Quite a few patients with limited or diffuse scleroderma will experience skin ulcerations on their fingertips, knuckles, or creases of their fingers. This can also occur on the toes. An open area can persist for months, so vigilance is necessary to minimize its impact.
Here are a few tips to help prevent ulcerations and to care for these areas if ulcers develop.

• Prevention is key.
• Protect your hands from trauma. A small scratch or cut can lead to ulceration.
• Practice good skin care. Moisturize often, particularly after washing hands; protect skin from cleaning detergents by wearing gloves; avoid hot water; and never pick or dig at calcium deposits.
• Keep warm. It is important to keep your entire body warm, not just your hands. During cool or cold weather, wear layers to keep your trunk warm. Wear a hat and don't forget proper attire for your feet. You may want to consider the added benefit of chemical hand or toe warmers. Always preheat your vehicle before driving. Your hands may need protection in warm weather months as well. Wear gloves in the grocery store if you are prone to Raynaud attacks when exposed to colder sections of the store such as the freezer or produce and meat coolers. Use coozies over bottles or glasses that contain cold beverages, or decant your beverages into insulated cups.
• Try to minimize stress as much as possible, or identify techniques than can help you manage it.
• Do Not Smoke! The nicotine in cigarettes decreases blood flow to the fingers and toes. Second-hand smoke can also be problematic for those with Raynaud phenomenon.
• Avoid caffeinated beverages. Caffeine will also constrict blood vessels decreasing blood flow to the fingers and toes.
  
  If an open area develops, the following tips may be helpful:
  
• Keep the area warm to maximize blood flow.
• Keep the area very clean by using bandages.
• Soapy washes combined with topical antibiotics such as Neosporin or Bacitracin several times a day can minimize the risk of infection.
• Always report any new open area on your fingers or toes to your physician when you visit. If the area develops signs of infection that include redness, heat, excessive pain, drainage or you develop a fever please contact your physician for care. An oral antibiotic and close monitoring by your health care practitioner will be necessary to treat the infection and manage healing.
• Pain control is very important while managing a digital ulcer. Uncontrolled pain can interrupt sleep, which in turn will increase pain, etc. It is a vicious cycle and has a huge impact upon daily life and ability to function. Please talk honestly with your physician about your level of pain and its impact on your life.
• Remember to take all medications prescribed for your Raynaud's. Good wound care and vasodilatation is the main-stay of wound management.

Itchy Skin (Pruritis)

Itching is a common problem for many with scleroderma. It is caused by an inflammatory process associated with the disease. This complication usually occurs within the first two to five years of the disease and then will subside. For some, the problem may persist.

Unfortunately, it can be difficult to manage; but here are a few hints that may help you.

• Remember to moisturize your skin regularly, particularly after bathing. Use of a product that contains lanolin is recommended. Dry skin can intensify the itch.
• Avoid hot showers or baths that rob the skin of needed moisture.
• Use gentle soaps such as Neutrogena or Dove. Non-soap cleansers such as Cetaphil or Aveeno may also be used.
• Use a humidifier in cold winter months to help keep skin moist.
• Experiment with moisturizers until you find one that is compatible with your skin type. Many patients have reported going through several until they found the right product.
• If the above measures are not enough to manage your pruritis, you may want to discuss prescription options with your physician. Antihistamines such as Benadryl and Atarax have been helpful, as have some topical hydrocortisone creams. Some physicians have found colchicine effective. In severe cases, low-dose prednisone has been used.
• Try not to scratch. Scratching can increase the itch, and potentially cause breaks in the skin that may become infected.
• For some, choosing to wash clothes in a gentle detergent like Dreft or Ivory Snow may be helpful, in addition to avoiding fabric softeners or dryer sheets.
• Others have indicated that 100% cotton clothing is more comfortable against their skin than blends, synthetics, or woolens.

Telangiectasia

Telangiectasias (telan-jee-eckta-zhas) are tiny red spots caused by the dilatation (widening) of small blood vessels in the skin. They appear as reddened freckles on ones face or hands.

Some persons experience telangiectasias on their lips and inside their mouths. These lesions are benign and do not pose any danger. However, many find them to be cosmetically unacceptable, and may choose one of the following methods to improve their appearance and feel better about themselves.

Over-the-Counter Products to Cover Telangiectasias

There are wonderful cosmetics available at department stores, dermatologists' offices, or on the Internet that very effectively conceal the affected areas.

Two of the most popular products reported by patients as easy to use and effective are Dermablend corrective cosmetics and Coverblend by Neostrata.

Laser Treatment to Remove Telangiectasias

Laser treatments can effectively remove telangiectasias for those who desire a more permanent approach. This treatment is usually not covered by insurance because it is viewed as cosmetic. If you consider this procedure, always work with a dermatologist or plastic surgeon who is board-certified and has had formal training in the use of lasers.

It is important to note that telangiectasias tend to recur.

In rare cases, telangiectasias can occur in the throat or stomach and cause bleeding. This requires immediate attention. Gastroenterologists can successfully treat these areas with laser therapy.

Fingernails and scleroderma

Some persons with scleroderma will report a fingernail that appears to be separating from the nail bed. This is called onycholysis, and can occur due to Raynaud phenomenon or systemic disease such as scleroderma.

Patients are advised not to trim or push back their cuticles. However, if you decide to do so, be very careful not to traumatize the surrounding tissue, which could lead to a non-healing ulceration or possibly infection.

If you visit a nail technician for your nail care, always educate them about your scleroderma and Raynaud phenomenon. They will need to be gentle and vigilant so they do not nick or scratch your fingers or toes.

The few minutes it will take to educate the service provider can save you weeks or months of discomfort.