Caregivers' Guide: Section 3
General Advice about the Skin
In general, the goal in taking care of a child with ichthyosis is to hydrate (moisturize) the skin, hold in the moisture, and keep scale thickness to a minimum. But remember – ichthyosis is different from person to person. Two people with the same type of ichthyosis may have very different expressions of the condition and their response to the similar treatments may be very different as well. What works well for one person may not work well for you or your child. For instance, a lotion that works wonderfully for one child may be painful and even harmful for your child.
Your dermatologist and pediatrician are, of course, excellent sources of information. But don’t forget a third professional - the pharmacist. A good pharmacist can make life much easier for a family dealing with ichthyosis. He or she can offer suggestions, keep you informed about new products, place special orders for you, and offer you discounts for products you buy in bulk.
In time, you will become the expert in the practical care of your child. You will develop your own regimen by consulting with doctors, talking to other parents of children with ichthyosis, and experimenting on your own. You can keep abreast of what other people are using by following FIRST’s quarterly newsletter, Ichthyosis Focus, and by interacting with other parents through the Regional Support Network or Facebook Group Page for Parents. Always check with your doctor before changing your routine or trying a new product, even if another parent swears by it.
In the following sections, we have tried to offer some general guidelines on skin care. These are only guidelines. You, your pediatrician, and your dermatologist should ultimately design your child’s skin care regimen.
Baths are good; more baths are better. Despite popular belief, dry skin does not lack oil, it lacks water. Baths put the water back into the skin, soften scale so it can be scrubbed off without too much difficulty, and are also great splashy playtimes for you and your little one. Keep in mind that soaps rob skin of essential oils, so they should be used sparingly or avoided altogether. Speak to your dermatologist about how much soap you should use. Some types of ichthyosis respond better to the steam in a comfortably warm shower than to complete immersion in a bath. Experiment to see what works best for your child.
You might also consult your dermatologist about which bath additive might be best for your child’s skin. Doctors often recommend oatmeal-based additives, which you can buy at the grocery store or drugstore, or make at home if you have a food processor to finely grind whole oats. For patients who are at risk for infection, doctors sometimes recommend adding a few drops of antibacterial soap or even bleach (e.g. Clorox®) to the bath. Although the precise measurement of the mixture varies from person to person, it is recommended to use approximately 1 to 2 teaspoons per gallon of water. Also, some shampoos can help reduce scale on the scalp.
If your baby has large raw areas on his or her skin (as many babies with EI do), put a dab of petroleum jelly on the raw areas before the bath. This helps alleviate the pain of the first contact with the bath water.
Once your child has had time to play and soak, scrub the loose skin off gently with a your palms, a wash cloth, or a specially made product designed to loosen skin cells. (Nail salons often carry several kinds of nail filing blocks that are gentle to the skin.) You can also try a loofah or a pumice stone if your child has thick calluses. You will learn with experience how much to scrub. Moderation is the key. If too much skin is removed, there will be tender (sometimes very red or even raw) skin beneath. It is especially important not to overdo scrubbing off the scale in babies. Some topical medications may be absorbed internally if the skin is overly abraded or raw. Excessive scrubbing can also cause blisters in children with EI. And scrubbing of any type is not recommended in Netherton syndrome.
After a bath, thoroughly dry the creases under the neck, under arms and in the diaper area to guard against maceration, which are sores resulting from over-moisturized skin in creases and folds. Only pat dry the rest of the body to keep the skin moist. Immediately apply cream or lotion to seal in that precious moisture.
How often should you give your child a bath? It depends on the condition of the skin and the weather. If you live in a climate with dry, windy, or cold weather, you may have to give your child more frequent baths. If your child is in air-conditioned environments, he or she may also need more frequent baths.
Cream, Lotions & Ointments
You will want to set up a convenient, colorful, and well-lit area in your home to apply lotions and creams. At first, you may need to coat your baby at every feeding and/or diaper change. With time, though, you will learn by experience what your child’s skin needs. Babies will often react better to warmed cream or lotion, as opposed to cold lotion. Try rubbing a scoop full between your hands before applying the cream or lotion on your baby. Or try putting the tightly closed container in the warm bath water while you bathe him or her; the lotion will be warm when the bath is done. Next, moisturize the rest of the child’s body. Rub the lotion or cream in well, gently massaging your baby as you go. This can be a nice time for telling stories, singing songs, or just talking to your baby.
Some of the most effective agents to remove scale include alpha-hydroxy acids, such as lactic or glycolic acid. Some products containing these ingredients are available by prescription, although many over-the-counter products contain them as well. Remember, though, that sometimes it is better not to try to remove the scale, but just to keep the skin supple and well lubricated. Glycerin, urea, and propylene glycol are other effective ingredients that attract water (called humectants) and work well on severely dry skin. Glycerin alone can be purchased from any pharmacy; you can add it to your favorite lotion to help the skin stay moist longer. Skin barrier repair formulas containing ceramides or cholesterol may also improve scaling.
Use your dermatologist’s guidance, but at the same time begin learning the active ingredients in the creams and lotions you use. Although there is a dizzying array of topical products available for purchase over the counter, most, you will find, are composed of a relatively small number of key ingredients. At the same time, prices vary considerably, even among remarkably similar products.
Often, a heavier cream, which forms an occlusive barrier, is helpful to seal in moisture once you have applied a humectant. Again, these are most effective when applied immediately after a bath. Many occlusives contain a petrolatum or mineral oil base. Lotions, although easiest to apply, are often relatively ineffective at holding moisture in the skin.
Some products (particularly the alpha-hydroxy acid lotions and creams) may cause stinging or mild irritation especially in small children, so it is wise to discuss their use with your dermatologist. Avoid applying these to areas where the skin is cracked or raw. Furthermore, there is a theoretical risk of systemic (internal) lactic acidosis (accumulation of lactic acid in the blood above normal levels), particularly in infants. Again, work closely with your dermatologist in determining your child’s treatment regimen.
Diapers & Clothing
Your choice of diapers may depend on what type of ichthyosis your child has. Disposable diapers, particularly the super absorbent type, usually work well on children with ichthyosis (except those with EI). They pull most wetness away from direct contact with the skin, yet are occlusive, so they hold in moisture. Because of this, the skin in the diaper area may look better than on other parts of the body.
Babies with epidermolytic ichthyosis (EI) often cannot wear disposable diapers because they are too rough on the skin. All-cotton diapers don’t wick away moisture, but without consistent use of plastic pants, the moisture will evaporate and the cotton will be the least irritating. To counter the moisture loss, use petroleum jelly or similar occlusive often, and rub it in well. Thickly grease the diaper area to prevent urine or feces from contacting your infant’s skin, especially if he or she has deep, open cracks or raw patches.
If your EI baby has extremely fragile skin, he or she may not be able to wear diapers or clothing at all without blistering and peeling. In this case, buy or make oversized cotton receiving blankets. In the center of one, place a waterproof lap pad (available at baby stores) covered by layers of cotton diapers. Put your baby on top of the diapers without pinning them, and wrap him or her in a loose swaddle. (In the summertime, don’t even bother to swaddle. Just place your baby on top “au natural.”) If your baby still cannot wear diapers when he or she begins to roll over or crawl, make the lap pad and diaper layers bigger in the playpen. You will go through a lot of receiving blankets and diapers, but the mess will be minimal, and your baby will do better without the irritant of fastened clothing.
Ask your dermatologist about the best method of cleaning your baby at diaper changes. Some recommend unscented diaper wipes without alcohol, cotton balls covered with oil, or just plain water. Another mom suggests placing a roll of toilet paper into a large plastic bag into which a small amount of baby oil has been added. The toilet paper will absorb the oil, and the plastic bag makes the whole ensemble easy to carry in a diaper bag.
Buy clothes large, so they do not rub. If your child’s skin is especially sensitive, turn outfits inside out to avoid having the seams rub. 100% or mostly cotton clothing is the least irritating. Avoid elastic. Avoid polyester, especially acrylic sweaters, since it can irritate the skin. Take your child with you while shopping and rub his or her hand on clothing you are uncertain about. If it catches, do not buy it. Babies and young children tend to scratch themselves until they are raw and bleeding. One piece footed pajamas, outfits, or “onesie” type shirts with overalls tend to prevent scratching to rawness or infection in children under age 3. Most babies, whether they have ichthyosis or not, need nothing on their feet, which can be especially sensitive. This will change once they begin walking, as walking barefoot dries out the feet and pressure from walking can cause cracks in thicker calloused areas such as the heel. Wearing shoes and socks helps keep the feet hydrated and reduces the depth and frequency of the cracks.
Using a heater and a humidifier may keep the room warm enough so clothes will not be needed. Also, putting your baby on a lambskin pad in the crib or playpen will help him or her maintain body temperature while providing extra soft padding.
People with ichthyosis have an impaired skin barrier function that leaves them more susceptible to infection. Thick moist scale will trap bacteria, yeasts, and fungi. The cracking and fissuring of the skin also leaves the body open to infection. Your dermatologist can help you watch for fungal, yeast and bacterial infections, and teach you how to distinguish between them. All infections require prompt medical attention and can usually be treated with topical prescription ointments and creams.
If your baby’s skin is open or raw, begin with applying the antibiotics suggested by your dermatologist to these areas. Infections may begin as raised white bumps, raw areas that are weeping, or areas covered with a yellowish crust. If your child seems particularly itchy and uncomfortable, or has a low fever without any other obvious symptoms, he or she may have an infection. If your child develops a high temperature, or if the infection spreads, contact your doctor immediately. Treatment with either oral or parenteral (intramuscular or intravenous) antibiotics may be needed. This is particularly important in newborns, in whom infections can spread rapidly and can be serious.
Nutrition & ichthyosis
Because the skin is the largest organ of the body and because it has a very large surface area in contact with the environment, what happens to the skin has a significant impact on the whole body. It is important to realize that the skin requires large amounts of iron, minerals, and particularly protein to continue functioning. Increased loss of skin from the surface puts an enormous strain on one’s entire system.
Children with hyperproliferative types of ichthyosis, such as widespread Netherton syndrome, EI, and CIE, where there is rapid skin turnover, are at most risk for growth retardation. The nutritional demands are very high for these skin types and are even higher in children who are growing rapidly to begin with. Many children with ichthyosis consume enough calories and nutrition to support normal growth, but they do not consume enough calories to compensate for the increased demand of their ichthyosis. Infants with ichthyosis may need supplemental feedings and nutritional supplements to support their growth. Consulting with a nutritionist may be helpful.
Children with poor skin barriers need foods that are adequate or even “super-adequate” in their protein and iron content just to maintain “normal” growth. Some children with ichthyosis may be smaller than other siblings in the family, which may relate, at least in part, to their nutrition. Thus, a good diet and supplementary vitamins and minerals are important.
Infants with severe forms of ichthyosis may be born prematurely and are small for their gestational age, and when nursing they may suck poorly due to restriction of the inflexible skin around the mouth. The breast, of course, is flexible to the mouth of the infant and is therefore preferred. Failing this, a preemie nipple (one that is more flexible and doesn’t require a strong suck to deliver) may be used with the holes slightly enlarged to better deliver milk. Look for nipples labeled “Neonatal,” “Premature,” or “Natural Flow.” Again, breast milk is high in minerals, vitamins, and protein. It is important for mothers to maintain good nutrition so infants will get the highest quality milk. Of course, if formula is used it should be one enriched with iron.
Solid foods should probably be introduced at the normal time, at four to six months of age. Enriched baby cereal is an excellent first food because the protein in the cereal is the best absorbed of all suitable infant proteins. When your baby is ready for more texture, try whole grain cereals.
Many nutritionists feel that children should not be subjected to adult “diets” that are low in cholesterol and fats because children need the cholesterol and the nutrients in all dairy products, and in eggs, for normal body and brain growth. This is particularly true for children with ichthyosis who need whole milk, especially during the growing years.
Managing your child’s diet on top of round-the-clock skin care is not easy. Your child’s pediatrician or a nutritionist can help you develop a balanced diet plan that is nutritionally and developmentally appropriate for your child.
If you are not already aware of which foods are high in iron, protein, vitamins, and minerals, excellent books are available at many libraries and bookstores. Nutritional services may be available at agencies in your area.
While not all children with ichthyosis experience growth problems, a significant number do. If your child with ichthyosis begins to experience these problems, have your child evaluated by a pediatrician. Consider the following helpful hints:
- Try to keep a written record of everything your child consumes each day. Feed infant cereal until your child is at least 12 to 18 months, since it is the best absorbed of all infant-suitable proteins.
- Offer nutritious and interesting meals and snacks.
- Respect your child’s ability to know when he or she has had enough to eat. Offer plenty of liquids, since water is lost through the skin. This is especially important when your child has diarrhea or is vomiting.
- Don't let meal times become burdensome or stressful. Keep feeding times pleasant and relaxed.
- Try planning a daily eating schedule, which might include three meals and three snacks. Keep plenty of healthy snack choices on hand
Children with ichthyosis are more vulnerable than most children to temperature and the weather. Overheating can become a significant problem since the child’s thickened skin does not allow the body to get rid of body heat through sweating. During hot weather and when your child is active at play, watch carefully for signs of overheating. The skin may redden, your child may appear cranky, and, when seriously overheated, your child may become listless and lethargic. Overheating is a potentially dangerous situation, and steps must be taken immediately to bring your child’s core temperature down to normal levels. Take him or her into a cool environment and, if necessary, put him or her into a lukewarm bath. Applying wet, cool washcloths to the face and extremities may also be helpful if the overheating is not yet too serious. If you are seriously concerned about how overheated your child is, call 911.
The best treatment of overheating is prevention. Make ice water or juice available all the time. A spray bottle of cold water is useful for counteracting the effects of hot weather, for example when riding in a car. A game may be made of spraying with cool water. For outside play during warm weather, one mom dresses her child in wet clothes. Available, too, are “cool vests,” which are pocketed smocks in which packs of frozen coolant gel are placed. In excessively hot weather, your child may need to stay inside.
If your child spends time in preschool or with any outside caregiver, the person must be aware of the signs of overheating, and must be able to respond to them at once.
When overheating is due to fever, try to keep the fever down with an aspirin substitute (for example, acetaminophen, or Tylenol); you may also use lukewarm baths to cool your child. During a fever, avoid thick creams that can hold in the heat. Many parents have noticed that after a high fever, their child’s skin takes a turn for the worse. This is a temporary setback, but may require extra attention for a few days.
To help avoid overheating due to warm weather, dress your child in layers that can be removed as needed. When out in the sun, use a hat with a wide brim to protect both eyes and skin. Ask your doctor about sunscreens.
FIRST publishes a resource sheet on overheating that details the signs and symptoms of overheating, what do to in a heat emergency, and resources for cooling products and sun protective clothing. Parents may request this resource sheet and copy it to all the individuals who help care for their child.
Systemic therapies: The retinoids
Some severe cases of ichthyosis have improved when treated with oral retinoid therapy. The retinoids are a class of pharmaceutical product available only by prescription, and a physician who is experienced in their use must monitor their use closely.
The key retinoid drugs, Soriatane® (acetretin) and Accutane® (isotretinoin), are derivatives of vitamin A, which in its natural form is toxic in high doses. Although these synthetic derivatives of vitamin A substantially reduce the natural toxicity, they still remain potent and potentially dangerous drugs.
Because retinoids may cause significant effects on bone development, their use in children who are still in their growing years requires careful consideration. Regardless of age, periodic X-rays to monitor bone development are essential. Other side effects include fluctuations in body chemistries (for example, cholesterol, serum triglycerides, and liver function), so retinoid therapy requires a physician to closely monitor the patient, completing essential tests at regular intervals.
Most significant, perhaps, is that the retinoids are highly teratogenic, that is, their presence in the system of a pregnant woman has a high likelihood of causing major birth defects. Even one pill of an oral retinoid can cause birth defects. Women taking oral retinoids must discontinue use of the drug for several months to several years, depending on which retinoids they were using, before attempting to become pregnant.
The beneficial effects of retinoid therapy on the skin will last only while the drug is being taken. When therapy is discontinued, the skin will revert to its previous condition. Therefore, the retinoids are considered long term therapy. Given the dangers associated with their use, one must weigh carefully the advantages and disadvantages. This is particularly true for women of childbearing age and children.
Anyone using retinoid therapy must have a close working relationship with a physician who has sound experience in using these drugs with ichthyosis, and with whom the patient has both trust and confidence.
Dressing wounds on babies with EI When EI babies have wounds that must be dressed, a non-stick bandage pad can be held in place by the soft cotton wrap used under casts, or gauze wrapping available at most drug stores. Wrap the gauze around the entire extremity. Regular stick-on bandages do not stick to EI skin. If they do, they often rip off healthy skin when they are removed. If your child is hospitalized, oxygen monitors and IVs cannot be held down directly with surgical tape. Have nursing staff wrap the arm with gauze, and then tape the IV to the gauze, not directly to your child’s skin.
Watching for infection Your dermatologist can help you watch for fungal, yeast, and bacterial infections, and teach you how to distinguish between them. All require prompt medical attention and can usually be treated with topical prescription ointments and creams.
Itching Itching can be treated with oatmeal and oil baths and plenty of cream or lotion. If the itching persists, your doctor can prescribe oral and/or topical medications.
Scalp Scale To remove difficult scales on the scalp, try applying your favorite lotion liberally to the scalp, then put on a shower cap or other occlusive garment for the night. In the morning, shampoo, massage, and comb out.
Scratching and Fingernails Infants with ichthyosis often have fast-growing nails. Take care to keep them trimmed below the fingertips. You might want to try adult cuticle nippers instead of baby nail trimmers. Babies tend to scratch the upper arch of the ear, behind the ears, and their noses. You can put socks on the hands of your sleeping infant, but they learn to take them off after a few months. Leaving your baby’s hands covered all the time could delay development of hand-eye coordination. If your baby seems to be scratching a lot, he or she may need an extra bath and lotion to feel comfortable, or they may need to be evaluated by your doctor for a skin infection.
Ear Wax and Scale Just as the skin sloughs off on the areas you can see, it also sheds inside the ear where it can combine with wax and form a solid material that can smell bad and temporarily impair your child’s hearing. Remember that infants must hear well to learn to speak well. Have your child’s hearing checked regularly and discuss with your pediatrician safe ways to keep your child’s ears cleaned of debris from exfoliating skin. Your physician may recommend an earwax remover, sold over the counter. Never stick anything in your baby’s ears; this can puncture the eardrum and cause hearing loss. Some ichthyosis patients get relief from pure lanolin. Lanolin is easier to spread when mixed with water.
You can safely clean the arches of the external ear and also behind the ear with a cotton swab. Keeping skin growth under control in these areas will reduce itching and resulting open sores. If your baby seems to constantly rub at or pull at his or her ears, he or she may have a build up of scale in the ears that is causing itching.
It takes time Give any new cream or other treatment a decent trial before deciding it doesn’t work, and don’t expect miracles. Some products need to be applied for at least two or three weeks before results become noticeable. If you are comparing treatments, use the regular treatment on most of the body, and apply the new treatment to a small area such as the thigh or forearm only. This way, you can see a visible difference between the two. If the new treatment is ineffective or causes the skin to blister, itch, peel, or dry out, the problem will be limited to the small area.
Body odor Many people with ichthyosis have a distinctive, sometimes unpleasant, body odor. The problem is usually caused by bacteria trapped in thickened skin or in cracks or creases between thickened scales. It is usually most noticeable in the creases in a baby’s neck or in the clenched hands. Try bathing with an antiseptic skin cleanser, or adding two teaspoons of chlorine bleach to each gallon of bath water. Consult with your dermatologist for specific recommendations and amounts to use.
Ectropion Infants and children with ectropion (eversion or flipping out of the eyelids) may have difficulty with dry, irritated eyes. Many children with ichthyosis sleep with their eyes open. Watch out for a child who rubs his or her eyes, or has frequent scratches around the eyes. Pay attention to pink, puffy eyelids or bloodshot eyes even when the child is not tired or sick. See an ophthalmologist regularly in order to guard against corneal damage that may result from dryness. Your physician may recommend drops or ointments to keep your child’s eyes moist.
Laundry Care Creams and ointments can wreak havoc when it comes to caring for your child’s clothes. Laundry products free of dyes and perfumes are preferred over most products. The “NO” list includes fabric softeners as well as detergents with heavy fragrance and other chemical additives, as they can make skin itchy, red, and uncomfortable. All Free & Clear® is free of perfumes or dyes, and can be less irritating to the skin. You’ll find a Laundry Care Resource Sheet with lots of tips from experts and other members available from FIRST.
This information is provided as a service to patients and parents of patients who have ichthyosis. It is not intended to supplement appropriate medical care, but instead to complement that care with guidance in practical issues facing patients and parents. Neither FIRST, its Board of Directors, Medical & Scientific Advisory Board, Board of Medical Editors, nor Foundation staff and officials endorse any treatments or products reported here. All issues pertaining to the care of patients with ichthyosis should be discussed with a dermatologist experienced in the treatment of their skin disorder.