To preserve and protect the health of and to relieve persons affected by ichthyosis and any associated condition.
To enabled the basic knowledge of medical professionals and the general public on the subject of ichthyosis and its implications for the family.
To promote research into the management of ichthyosis and to publish the useful results thereof, and to support organizations promoting research into ichthyosis.
CORE VALUES
Compassion – We are a caring organization recognizing the unique challenges faced by our community and will provide support with kindness and empathy.
Hope – While celebrating today’s strengths and successes, we strive to convey to all individuals affected by ichthyosis that the future will be filled with friendship, support and a cure for ichthyosis.
Integrity – We will conduct ourselves in a trustworthy, ethical, and reliable manner in everything we do and say.
Responsiveness – We provide accurate and timely information about ichthyosis to meet the medical, social and educational needs of our community.
This is the commonest form of inherited ichthyosis, affecting 1 in every 250 people. Ichthyosis vulgaris has been found to be due to a gene defect in filaggrin, which is a protein in the skin that impairs the skin barrier formation and the natural moisturising factors that are key to keeping the skin hydrated. It is usually quite mild and develops in early childhood with fine, light grey scales and roughness on the upper and lower limbs, but sparing the folds of the arms and legs. It may be more widespread and is more obvious in the winter time. It is sometimes associated with atopic or childhood allergic eczema and may cause an increased wrinkling of the palms and soles.
It can be treated with regular application of moisturisers. It improves in adult life and may be passed on to children whereby there is a 50:50 chance of each child having the disease (“autosomal dominant transmission”).
X-linked ichthyosis
This condition occurs in males only and develops in infancy with tan or grey scales on the limbs and across the trunk. It may affect the ears and face and the scales appear to be stuck on like stamps. It varies in its severity and improves in fine or sunny weather. It changes very little with age.
X-linked ichthyosis is due to lower levels of an enzyme known as steroid sulphatase. This condition is passed on by a mother, who is a carrier of the abnormal gene, to her son with a 50% risk for each son. A carrier mother shows no evidence of the condition. The gene that causes this condition has been identified and, very rarely, a similar fault can affect adjacent genes on the same chromosome, causing a variety of other problems for an affected male, for instance, bony defects or lack of the sense of smell.
A mother who is carrying an affected male baby may have a prolonged and consequently difficult labour. A small number of affected boys have poorly descended or undescended testicles; it is usual to check this aspect of development in affected families. The child’s growth should be monitored from time to time. It is quite common to have asymptomatic specks in the cornea that do not interfere with vision which are identified by an eye specialist.
Autosomal recessive congenital ichthyoses
There are three types of ichthyoses in this category: Congenital ichthyosiform erythroderma, Lamellar ichthyosis and Harlequin ichthyosis. These three conditions are passed on by parents with normal skin who both carry the abnormal gene (autosomal recessive) and the risk of a further child being affected is 1 in 4.
Congenital ichthyosiform erythroderma
This type of ichthyosis generally show signs at birth with the appearance of a collodion membrane on the newborn baby. This is a shiny yellow film stretched across the skin like a sausage skin. It dries out and gradually sheds within the first week of life. The “collodion baby” is nursed in a humidified incubator until the skin settles down. The majority of collodion babies will develop congenital ichthyosiform erythroderma. However, a small number of these babies will have normal skin once the membrane is shed and, therefore, it is not possible to accurately predict the outcome in the early stages.
Most collodion babies develop non-bullous ichthyosiform erythroderma, which literally means inflamed, scaly skin, without blisters. It affects 1 in every 300,000 births and so is very rare. Once the collodion membrane has shed, the skin remains red and has fine, white scales affecting the entire skin surface. In severely affected children the eyelids may be pulled outwards and there may be some mild scalp hair loss and tightness of the fingers. Because the skin is inflamed it will feel hot, even if the child is cold. Most children with this condition do not sweat normally and may overheat in hot weather, when exercising or with a fever. The palms and soles are sometimes thickened and scaly. Otherwise a child’s health is normal. They may suffer cosmetically and this is especially important when a child starts school, or in the teenage years.
Lamellar ichthyosis
Lamellar ichthyosis is a rare form occurring in 1 in 200,000 live births. Newborns usually present with a collodian membrane but the skin is different in that it is less red but the scaling is larger, perhaps darker and more adherent, or stuck down.
Harlequin ichthyosis
Harlequin ichthyosis is a very severe, but extremely rare type of inherited ichthyosis (approximately 5 per year in the UK). It is evident at birth due to the very thick scaling all over. Intensive care is required and detailed information on the condition will be needed for the parents and staff.
Bullous ichthyosis
Bullous ichthyosis also known as bullous ichthyosiform erythroderma, is another rare inherited ichthyosis. At birth the baby’s skin seems to be fragile and may show blisters, without much scaling. This causes severe problems for the young infant and intensive care is sometimes necessary in the first few weeks of life. A skin biopsy will be required early on to confirm the diagnosis. During the first year or two of life, the blistering tendency reduces but widespread redness, scaling and thickening of the skin becomes more obvious through childhood. This produces warty skin changes around the creases of the joints. Skin infections are quite common and can lead to a characteristic odour. There may be a reduction in sweating in childhood, which improves later in life. This is a troublesome and distressing condition for the child and the family.
It is transmitted as an autosomal dominant disorder, which means that one of the parents may be affected. However, in at least half of affected children, neither parent is affected; therefore the child has developed a new gene fault while growing in the womb. This means that there is no risk above the ordinary for further pregnancies in that family although the child may pass on the condition onto the next generation. Detailed genetic counselling is necessary.
Netherton’s syndrome
The incidence of this condition is not known but it is probably in the region of 1 in each 200,000 births in the UK. The newborn child is very red and has scaly or peeling skin. The infant is often underweight and slow to grow and this problem continues for the first year or two of life. The affected child may need prolonged hospital treatment until both the skin and the nutrition improve. A characteristic feature of Netherton’s syndrome is thin fragile scalp hair in the baby. Later it is spiky and this is an important clue to the diagnosis. In many affected children the skin improves in childhood, although it can flare up without warning.
Netherton’s syndrome is an autosomal recessive disorder where both parents are carriers and show no sign of the condition. There is however a risk to further babies of the order of 25%.
There are a number of other genetic or inherited forms of ichthyosis where there are other medical problems but these are so uncommon that they are not discussed here.
Babies born with ichthyosis may have a mild to severe case of ectropion. Ectropion is a condition whereby the skin around the eyes pulls tightly causing the eyelids to slightly turn outward,which is commonly seen in cases of collodion baby or harlequin Ichthyosis. If present, consult with an ophthalmologist. Infants with ectropion are at risk for developing keratitis, or inflammation of the cornea as the tight skin of the eyelids make it difficult for them to shut the eyes sometimes. So ensuring that the eyes are adequately lubricated is essential. This can be accomplished with hourly artificial tears or ocular lubricant. Vaseline can be applied around the eyes couple of times in a day or before going to sleep at night so that eyes can be closed more easily. Topical antibiotics may be required for treatment of conjunctivitis or corneal abscess.
And although ectropion almost improves beyond the neonatal period, it can persist throughout life and medical consequences including keratitis, conjunctivitis, inflammation of the conjunctivitis and epiphora, accessing watering of the eye, can ensue.
As your child ages, if the ectropion persists, there are surgical procedures available to them, as well as topical retinoid treatments. Topical night time eye ointments can be applied after washing the hands & then wiping the eye area with a warm, wet washcloth. Consult with your physician and medical team before pursuing new treatment for your child.
The daily skin care treatment for ichthyosis requires using large amounts of emollients on the skin. This is great for the skin, but can be very harsh on clothing, as well as washers and dryers.
Below is collection of tips, provided by ichthyosis affected members themselves, including strategies and products for laundry care.
Laundry products free of dyes and perfumes are preferred over most products. Fabric softeners as well as detergents with heavy fragrance and other chemical additives should be avoidedas they can make skin, itchy, red and uncomfortable.
Laundry Additives, Stain Removers, and Tips
Shake out clothes prior to placing them in the washer.
Laundry stain remover products are available in the market to remove stains from clothing.
Soak clothing in vinegar prior to washing.
Soak clothes in Vanish OxiAction& hot water for one or two hours prior to a wash cycle.
Add a half-cup of dish detergent with standard amount of laundry detergent. Add to the washer as the tub is filling, then add the clothes. This creates fewer suds in the load.
Consider setting water level to full load, and add fewer clothes than the cycle can hold.
Water temperature desires vary. Cold water is less harsh on fabric fibers. If using hot water, be sure to set thermostat at 120°F. Higher water temperatures over 120°F can cause severe burns, please use caution.
Vinegar is an inexpensive way to help remove grease stains, and even odor, from your wash. Add a ¼ cup of vinegar to the load of laundry.
When using bleach, run an extra rinse of vinegar or consider using a vinegar/detergent rinse after washing.
Add Vanish OxiAction to wash cycle with detergent.
Use a regular wash cycle, not a soak cycle for sheets and towels.
Keep it Cool
Some moisturizers are petroleum based and can keep the heat from the dryer insulated in clothing. The heat combined with the moisturizer can create a “flammable” atmosphere. Be sure to give your clothing space and time to cool down after the dryer cycle is complete. Laundry can be hung to cool, folded immediately when it comes out of the dryer, or run the dryer on a cool down cycle.
Washing Machines: Use and Care
Standard front loader washing machines cannot sustain a large amount of suds, so it is difficult to add any other type of product, other than standard laundry detergent. Grease from emollients also tends to ruin the rubber gasket in front loaders. Larger, industrial machines, like those found at the laundromat, may be an option as they are adaptable to far more cleaning agents and hold much more water, which is better for removing grease and moisturizer.
Purchase a second washing machine to use for clothes stained and soiled with skin care product.
Fill the washer with ½ to 1 cup of cleaner such as dish detergent and hot water; shut it off for 30-60 minutes to start breaking down residue, restart to complete the full wash cycle.
Run an empty washer with two tablespoons of baking soda and two cups of clear vinegar.
Remember to scrub the inside ring.
Fabrics
Clothes can feel damp, uncomfortable and soiled when they are saturated by products such as Vaseline.So choosing the right fabrics, will not only be comfortable to wear, but may help avoid spending more money on clothing than necessary. Natural fabrics like cotton, hemp, linen and silk last longer than polyester or other man made fabrics.
100% cotton clothing is easier to wash, and much more pleasing to the skin. Polyester, spandex, and most sweater materials can be very uncomfortable.
Silk is also soft on the skin and you can even machine wash silk, as it is the most durable natural fiber – however machine washing silk will make the color slightly darker.
Some members wear 100% silk or 100% cotton long underwear under their clothing to protect clothing & help make the clothes to last longer.
Fleece sleepers for younger children can work well in the cooler temperatures, and will not absorb lotions.
Elastic vs. Velcro: Petroleum, found in Vaseline,and elastic have the same chemical composition, therefore one causes the other to break down. This process can wreak havoc on elastic waist clothing. Clothes with velcro or drawstring waists, rather than elastic will increase wearability.
People with ichthyosis often develop itching, or “pruritus,” in hot, humid weather or when overheated by exercise, sunlight, or by overdressing in warm clothes. The reason for this is that inability to sweat normally as excessive skin scale clogs the pores of the sweat glands and traps the sweat. The trapped sweat irritates the skin, leading to itchiness, redness, and occasionally small blisters.
Scaling conditions that show skin redness or inflammation often have associated itching. These conditions include: congenital ichthyosiform erythroderma (CIE), erythrokeratodermia variabilis (EKV), Hailey-Hailey Disease, Darier disease, Pityriasis Rubra Pilaris (PRP), and others. Sjögren-Larsson syndrome, although not characterized by inflamed skin, also tends to be itchy. In this case, the metabolic defect results in accumulation of molecules (leukotrienes) that can stimulate itching. Use of a leukotriene synthesis inhibitor, such as zileuton, may relieve the itching in Sjögren-LarssonSyndrome.
People with Netherton syndrome and Ichthyosis Vulgarismay also experience atopic dermatitis. Atopic dermatitis is a chronic inflammatory skin condition that is characterized by extreme itching, leading to scratching and rubbing that, in turn, creates the lesions of eczema. Another characteristic of Netherton Syndrome is a predisposition to allergies, such as asthma or food allergies.
Cold winter airwith low humidity, can worsen the dry skin of people with all forms of ichthyosis, which may also result in itchy skin. Itching can also be a symptom of secondary infection with bacteria, fungi, or infestation with scabies or lice. Itching is often worse at night, and can be made worse by tension and anxiety.
General treatment strategies for the itchy skin of ichthyosis :
1.Consult with your family doctor or dermatologist to rule out a bacterial or fungal infection. Infections should be treated with the appropriate antibiotic or anti-fungal agent.
2.Keep the skin as moist as possible by using moisturizers and keratolytics. (Keratolytics, products containing alpha-hydroxy acids, or urea, that help exfoliate the skin may be useful, but, because they are sometimes irritating, can also increase itching in some patients). Skin care products which contain oatmeal, oatmilk, or colloidal oatmeal may help to soothe itchy skin.
3.Try to resist scratching! Scratching can cause a thickening of the skin and can lead to infection.
For temporary relief of itching:
Apply cool compresses to the affected areas.
Try a lukewarm baking soda or oatmeal bath.
Stay away from heat and humidity, and wear loose cotton clothing.
Apply over-the-counter preparations for minor skin irritations, such as those containing pramosone or menthol. Calamine lotion can soothe itchiness, but tends to be quite drying.
Do not use products with “caine” in them, such as Benzocaine® or Lanacane® products, or products with diphendryamine (Benadryl) directly on the skin. These preparations can effectively act as a local anesthetic by numbing the sensory nerve endings, but are known to be “contact sensitizers,” which can lead to skin allergies and a worsening of inflammation and itchiness in some individuals.
For prolonged, chronic, or problem itching, your doctor may prescribe:
Oral antihistamines: Antihistamines prevent the release of histamines deep in the cells of the skin. Histamines are chemicals that may aggravate itch nerves. Certain antihistamines are often used to induce drowsiness and promote sleep, if the itching is disrupting sleep. Doxepine and hydroxyzine are sometimes prescribed to relieve itching. These compounds have a sedating effect and are also used to treat depression and anxiety. Other antihistamines do not have this effect, so speak to your doctor about which formulas are right for you.
Topical steroids or a steroid-free topical immunomodulator: Topical steroids, from over-the-counter hydrocortisone to prescription strength preparations, decrease the production and action of chemical mediators of inflammation, and may help to reduce itching. The new steroid-free immunomodulators are also effective in treating inflammatory skin diseases like atopic dermatitis; however, many people with ichthyosis have skin that more easily allows the absorption of applied medications. Steroids or the non-steroidal medications could be absorbed through the skin and into the bloodstream. These medications should not be applied to large areas of the body surface by ichthyosis patients for this reason. Check with your doctor to find out which formulas may be right for you.
Oral steroids: Cortisone and its derivatives are among the most effective anti-inflammatory drugs known. Long-term use of steroids can cause side effects such as changes in physical appearance, weight gain, irritability or depression. Treatment with steroids should always be kept at the lowest possible effective dose and should never be stopped suddenly. Oral steroid use must be monitored by your doctor.
Systemic retinoids: Systemic retinoids, which are derivatives of Vitamin A in a pill form, help to normalize the skin and reduce inflammation throughout the whole body. Systemic retinoids do not treat the itch specifically, but help to improve the ichthyosis that is causing the itch. Systemic retinoid therapy almost always causes side effects; most are usually mild and easily controlled. However retinoids can also cause severe birth defects if taken by pregnant women. Retinoids should never be taken except under the direction of a doctor, and under strict guidelines, as outlined by the FDA and the drug company. Under no circumstances should retinoids be taken by anyone other than for whom the drug was prescribed.
Anti-depressant agents: Tricyclic antidepressants have an antihistamine effect, induce sleep, and reduce itching. If itching is so severe that it causes sleep deprivation, irritability, and stress, and does not respond to other treatments, antidepressants can help to reduce the itch, scratch, itch cycle. Doxepin and hydroxyzine are sometimes prescribed for itching. These compounds have a sedating effect and are also used for treating anxiety and depression. These medications need to be used under a doctor’s supervision.
The itching associated with various forms of ichthyosis can cause much physical and emotional distress. The scratch, itch, scratch cycle can lead to serious skin complications, including infection, and can interfere with daily activities, relaxation, and sleep. It is important to work with your doctor to identify the underlying cause of the itching and to determine the most effective, least potent treatment. Treating the problem on your own with may make the problem worse and lengthen the amount of time needed to get the itching under control. See your doctor early to get the best possible treatment advice.
Overheating is a common problem for many people affected with ichthyosis as they can’t sweat normally particularly Lamellar Ichthyosis. Though they have sweat glands, the thickness of their skin and the scale does not allow sweat to reach the surface of their skin and cool them effectively. So they are at risk for overheating, heat exhaustion, and heat stroke in very hot and humid weather.
Strategies for avoiding heat emergencies:
Avoid physical activities and outdoor activities at the hottest parts of the day, from 10:00 a.m. to 2:00 p.m.
If possible, look for air-conditioned spaces during the hottest part of the day. If you cannot be in air-conditioning, use a fan to keep air circulating around you, stay out of the sun, and rest.
Drink lots of fluids. Avoid caffeine and alcohol, which can be dehydrating.
Use a pool, wading pool, lawn sprinkler, bathtub, or shower for frequent cooling dips.
Wear a wide brimmed hat and sun protective full sleeved clothing when you do need to be outdoors. Loose natural fiber clothing in light colors will help you feel cooler than dark, tight-fitting, synthetic fiber clothes.
Use cool packs, cooling scarves or other cooling products, and spray bottles of ice water to cool down.
Early Symptoms of a Heat Emergency
Dizziness
Fatigue
Muscle cramps
Nausea
Thirst
Feeling weak and light-headed
Late Symptoms of Heat Exhaustion
Cool, pale skin
Large pupils
Headache
Nausea and vomiting
Irrational behavior
Unconsciousness
Symptoms of Heat Stroke
Dry, hot and very red skin
Fever
Dark urine
Extreme confusion
Rapid, shallow breathing
Rapid, weak pulse
Small pupils
Seizures
Unconsciousness
First Aid for Heat Emergencies
Remove person from the heat to a cool place where they can lie down with their feet elevated about 12 inches.
Apply cool wet cloths (or cool water directly)to the person’s skin and use a fan to help the water to evaporate and lower their temperature. Place cold compresses on the neck, groin and armpits. Do not use alcohol rub.
Give the person cool water to sip, fruit juice or slightly salted water (1 teaspoon salt per quart of water). Give ½ cup every 15 minutes. Do not give salt tablets, alcohol or caffeine.
Seek medical help immediately if the person shows signs of shock (bluish lips and fingernails, and decreased alertness), has a seizure, or loses consciousness.
People with ichthyosis, who shed more than the normal amount of skin, the skin also shed inside the ear canal which combine with wax and form a solid material. This solid material may itch or smell and may cause a reduction in hearing. Cleaning ear wax is the most sensitive part. Flaky external ear is usual. Some adults put few drops of oil or favorite lotion & use bobby pin/cotton bud to remove solid wax. If your child’s ear is not cleaned properly, it’s hearing will be impaired temporarily. Infants must hear well to learn to speak well. Discuss with your pediatrician about safe ways to keep your child’s ears cleaned of debris from exfoliating skin. Have your, or your child’s, hearing checked regularly. Your physician may recommend an earwax remover that is sold over the counter. Never stick anything in your or your child’s ears; this can puncture the eardrum and cause hearing loss. If your baby seems to constantly rub or pull at his or her ears, it may have a build up of scale in the ears that is causing itching.
Ichthyosis is different from person to person. Two people with the same type of ichthyosis may have different expressions of the condition and response to the similar treatments may vary as well. What works well for one person may not work well for another. For example, a lotion that works wonderfully for someone may be ineffective or harmful for your child. Consult with your dermatologist and pediatrician for updated information. Also make a good connection with local pharmacist who will keep you informed about new products, place special orders or offer discounts. In course of time, you will be a master of taking care of yourself or your child. You can also develop homemade lotion recipe by consulting with doctors & patient support groups.
Medicines, lotions, clothes, special first aid things etc .
Products containing glycerine, urea, alpha-hydroxy acid, ceramide, vitamin E, petroleum jelly, paraffin (light liquid or white soft), olive oil (plain or extra virgin), coconut oil, jojoba oil, mustard oil seem to help individuals with ichthyosis. Some people get relief from pure lanolin, which is easier to spread when mixed with water. Some products containing these ingredients are available by prescription, although many over-the-counter products contain them as well. You can also place special orders to a good pharmacist nearby. You can make your own moisture mixture: Petroleum Jelly + Urea 10%- 20% + Soft Paraffin + Glycerin (small amount)
Applying lotion and creams to wet skin works better than putting them on dry skin. The lotion traps the moisture. Putting glycerin on wet skin prior to applying lotion also works good as the lotion stays longer.
Try adding a few ounces of pure glycerin or vegetable glycerin to your cream or lotion, and the skin will stay moist longer. Glycerin bought by the gallon is less expensive than smaller bottles.
After you buy a new cream or lotion, give it a reasonable trial. Sometimes a product needs to be used for two or three weeks before results are noticeable. It is safe to test the cream or lotion on a limited area of skin, like an arm or a leg. If it’s effective, doesn’t cause the skin to blister, itch, peel, or dry out then apply on whole body.
Alpha-hydroxy acid products may sting or cause mild irritation in small children, so it is wise to talk to doctors prior application. Avoid applying these to areas where the skin is raw. Petroleum jelly works good on cracked skin.
Avoid heavily perfumed products as those can cause skin irritation. Instead, a few drops of an essential oil to a cream can do wonders for the smell.
In cold seasons, you can put warmed cream or lotion on your child’s skin. Try rubbing a scoop full between your hands before applying the cream or lotion 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.
A pumice stone rubbed lightly on thickened skin may help loosen crust. Try this when the skin is wet and soft. You can also use natural loofah sponge which is cheap & effective. Soak the loofah in water to soften & use for scrubbing after you buy it from market. Shower gloves are also available in body shops.
To removescales on the scalp, apply your favorite lotion and occlude with a shower cap or plastic wrap to bed. A morning shampoo, massage and brushing with fine toothed comb may help. You can use mild strength shampoo, baby shampoo or pears/dove body wash to wash hair once or twice in a week. You can use a nourishing conditioner after hair wash. After washing & soaking hair with towel when the scalp is moist, spray glycerin & rose water on head. Scalp will stay moisturized. Try to look for hair care brands with high levels of natural ingredients. Avoid drying products, and products containing non water-soluble ingredients such as silicones. These can build up scales & shield off moisture even more. Sulfate free shampoos are better option in that case.
Be careful not to braid or twist the hair too tight, to avoid disrupting the hair growth.
Body odor can be a problem for people with ichthyosis. It happens when bacteria is trapped in the dead layers of skin. Regular bathing is helpful. Adding two teaspoons of bleach or vinegar to one gallon of bath water may help.
Bacterial infections can be a problem for some people with ichthyosis. Some individuals suggest adding two TEASPOONS of Clorox to one gallon of bath water.Carefully measure each gallon of water as you fill your tub and then mark the tub so that you can always fill with the same amount. For example, add 30 teaspoons of Clorox to 15 gallons of water.
More baths are better. Water helps make dry skin easier to remove and makes people with ichthyosis more comfortable. Moisturizing soap- DOVE, NIVEA, PEARS, OLAY, GOAT’S MILK SOAP are commonly used & available in market. Everyday use of soap will make the skin rough so better to use every alternate day or twice/thrice in a week. Some people find salt, besan, oatmeal, baking soda baths soothing. Be sure to apply lotion or cream immediately after gently pat the skin with towel. Don’t rub the skin with towel as rubbing will remove all the moisture & lotions will be not soaked effectively. You can find sugar scrub, apricot/walnut scrub, charcoal scrub products in supermarket to scrub off the scales while bathing. Some types of ichthyosis respond better to the steam in a comfortably warm shower than to complete immersion in a bath.
If you have problems with yeast infections in the groin area or feet, wear cotton underwear and cotton socks. Have your dermatologist prescribe the appropriate prescription medication for yeast or fungal infections. These problems need medical attention.
Thick scale can sometimes harbor so much infection, causing bad odors, that an oral antibiotic may be needed. Some people remain on low doses of antibiotics for long periods to control infection.
If you need to apply bandages, ask your pharmacist about some of the newest no-stick types.
Retinoids, a prescription oral medication, have provided some patients with dramatic improvement. These drugs need to be closely monitored by your doctor as there are serious side effects.
Ear canals can be a problem when they become clogged with skin and wax. Check your child’s hearing routinely by an ear, nose and throat specialist for ways to properly clean the ears.
If you have mild or severe cases of ectropion, apply Vaseline couple of times around the eye so that the eyelids becomes soft & eyes can be closed easily. Artificial tears, ocular lubricants & topical retinoids are also used to lubricate the eyes adequately but should be used after consulting with an eye specialist.
Natural fibers, such as wool or cotton, tend to wick moisture away from the body. You might consider cotton blends for clothing worn next to the skin. Some people with ichthyosis find rayon and other synthetics to be very irritating.
Air conditioning and heating can be very drying, especially for small children. Fresh air, fans and humidifiers may prove to be more comfortable.
Children and adults who cannot tolerate heat can try cooling suits and devices.
Itching is a common problem. The best way to reduce itching is to maintain a daily routine of bathing along with moisturizing. Avoid scratching directly with fingernails as it will make skin raw & there is a chance of infection. Rub with the flat surface of fingers instead of that. You can wear cotton hand gloves also while sleeping.
Sunshine can work wonders for ichthyosis, but too much sun is dangerous. Always use a good sunscreen on the exposed part of the body 30 minutes before going outdoors. To ensure maximum protection, repeat the application every 2 hours after continuous exposure to the sun, after swimming, physical exercise. A good sunscreen has some criteria – broad spectrum protection (protects against UVA and UVB rays), SPF 30 or higher, water resistant, hypoallergenic & non-comedogenic.
Some cosmetics can conceal skin problems such as excessive redness. Check with your local department store for special cosmetics.
Diet has huge impact on skin. Make sure to eat clean nutritious balanced diet everyday. Drink plenty of water (minimum 2 litre/ 8-10 glasses), avoid junk foods & carbonated beverages, alcohol & smoking.
Stress has adverse effects on skin. It aggravates skin problems. Impacts of stress can be reduced by certain techniques – adequate sleep (7-8 hrs), regular physical exercises, swimming, cycling, breathing exercises, yoga, meditation, indulging in hobbies, joining support groups or taking help from a professional therapist.
A thorough physical checkup should be done at least once in a year which includes – Complete Blood Count with Differential, Metabolic functions of liver & kidney (blood sugar, electrolytes, albumin), Thyroid function test, Iron profile, Lipid profile, Specific Nutrient such as Calcium & Vitamin-D level in blood. Imaging tests like ultrasonography, bone scan, X-ray can be done if the physician advises.
Home Remedies:
Hair Care: Soak a towel in a bowl of hot water for few minutes. Then squeeze the towel to remove water, hold both ends of the towel & wrap the hot towel around the head & leave it for 10 minutes. You can also apply warm oil on hair & scalp then do the hot towel treatment. It opens the pores on scalp & thereby helps reducing the scales & itching. Quality of hair also improves.
Skin Care: Take half teaspoonful of sugar, add few drops of milk/coconut oil/curd & mix. Apply the mixture on damp skin of face, neck & body & leave it for few minutes, then rub slowly & gently so that the scales come off. Then wash with normal water. You can rub a little grated potato with curd on skin to remove the scales too.
Sandalwood powder, besan (pulse powder), rice flour, multani mitti are also beneficial when applied as a paste.
Another trick is to take 2 egg whites, whisk it with olive oil, pour in a bottle & store in refrigerator (the mixture lasts for 1 week if refrigerate). Apply this mixture everyday night on skin before sleeping. Wash skin with water in the next morning when you wake up. This reduces wrinkles & fine lines.
In Ichthyosis the body’s ability to produce the outermost layer of skin, the stratum corneum, is defective. So there is traffic jam of cells on the skin’s surface which causes scaly skin.
The goal in treating the primary symptoms of ichthyosis is promoting exfoliation (normal shedding) of the skin’s outermost layer. Bathing is important to the shedding process. It not only cleanses skin of dirt and other external debris, it completes the natural process of desquamation, sweeping away dead epidermal cells.
For some form of ichthyosis affected people, frequent bathing is extremely important, as it is not only the best way, but the most natural way to remove dry scales and skin, and most importantly, more frequent bathing can ease the distress of ichthyosis, making day to day life much more comfortable.
Following bathing recipes can be used in a tub filled with one-third of water & soak the skin for 30 mins to an hour, as per your need. These baths should be followed by application of an emollient.
Salt Bath : The aim is to produce 3% solution which is less than the amount of sodium chloride in the ocean. This equals 1/4 pound per gallon of water – or 5 lbs in 20 gallons or 7.5 pounds in 30 gallons. Less salt may work for some, if you soak for at least an hour. This is safe to use every day. You can add pool salt to your pool too.
Baking Soda Bath: The aimis to have a pH of 7.9. The amount of baking soda to add may vary with the quality (pH) of your tap water. In most cases, adding one-third cup to a tub one-third full with water will raise the pH to 7.9. You can test the water after the baking soda is dissolved with pH indicator paper. You must soak for 45 minutes to one hour before starting to gently slough scale with a washcloth, loofah or other gentle mechanical desquamator. Baking soda baths can be used up to several times a week.
Antimicrobial Bleach Bath:Measure the amount of water usually put in the tub. Add 10ml (2 teaspoons) of household bleach per gallon – i.e., about 1 cup in a half-full tub (40-50 gallons). Soak for a minimum of 15 minutes – longer if you want to remove more scale. Bleach baths once or twice a week should be adequate to reduce odor and the frequency of infections; daily for one week may be necessary for someone currently infected. Undiluted household bleach is quite irritating so be careful how you handle it.
Post Bath Emollient: It is important to use an emollient just after bathing. If you have taken an alkaline bath (by using baking soda or bleach) it is a good idea to restore the natural acid pH of the outer layer of skin by using an emollient with a slightly acid pH, such as Lac-Hydrin or AmLactin.
(Ref: Leonard M. Milstone, MD,Yale University )
Pros & Cons of Bleach Baths
Many patients with ichthyosis, despite their best attempts at personal hygiene, become colonized by an overgrowth of bacteria, yeasts, or fungi which results in an unpleasant odor. When these same microbes try to reside on normal skin, they encounter a much thinner, and much more acidic stratum corneum. The pH of normal skin (pH 5.0 – 5.5) resists the growth of many microbes, while selectively encourages colonization by the microbes that normally occupy the niche of the stratum corneum. In contrast to the low pH of normal skin, the pH of ichthyotic stratum corneum rises to pH 6- 7 due to excess scale, inflammation, and a defective barrier. This elevated pH and the thickened stratum corneum favor the overgrowth of many microbes, including not only those that populate normal stratum corneum, but also pathogenic microbes, such as Staphlococcus aureus. In that case, bleach bath helps to:
Decrease Odor – Bleach baths reduce the amount of odoriferous colonization by potentially pathogenic microbes on the skin surface.
Decrease Infection – Bleach baths can decrease the risk of infection.
However, there is a second consequence of the elevated pH of ichthyotic skin, and the even more elevated pH of bleach. There are enzymes in the stratum corneum that are more active at an elevated pH, and whose activity can have two important consequences:
Separation of Cells – First, some of these enzymes degrade the connections that hold the cells of the stratum corneum together, causing these cells to separate from each other, and eventually to be shed from the skin surface. Obviously, for someone who has too thick a stratum corneum, bleach baths could prove to be an advantage. It should be noted, however, that there are no experimental or clinical data proving that bleach promotes desquamation.
Inflammation – Second, some of the enzymes that are more active at higher pH can provoke inflammation, because they activate and release pro-inflammatory cytokines, proteins that normally are stored inside stratum corneum cells and shed harmlessly. Currently, there are no studies that address whether the slightly alkaline pH of bleach baths increases inflammation in those who have inflamed skin. Surprisingly, there is one study showing that dilute bleach has the ability to reduce inflammation in an animal model of inflamed (not ichthyotic) skin. So in sum, we still have questions about the impact that bleach baths have on inflammation in our patients.
Most of us would agree that, taken together, the benefits of bleach baths in reducing microbial overgrowth, odor and infections far outweigh the uncertainties arising from its slightly alkaline pH. Yet none of us would see any advantage to prolonged exposure to the abnormally high pH of dilute bleach baths for patients with ichthyosis. Instead, it would seem prudent to follow one’s bleach bath with a generous application of a slightly acidic emollient, such as Lac-Hydrin or AmLactin, to help restore the skin’s natural acid mantle.
(Ref: Leonard M. Milstone, MD ,Yale University and Peter M. Elias, MD, UC San Francisco)
Travel Tips for Bathing
Traveling with children who have ichthyosis is challenging particularly when it comes to bathing.
Bring along a blow up baby tub. It’s easy to pack and you don’t need to use the hotel tub.
Bring a big Rubbermaid container to pack things, and then use it as a tub as well when you are at your destination.
Cleaning sponges and bleach wipes could also do the trick for a bath.
Bring antibacterial wipes with you when you travel. You can use them for cleaning stations and bathtubs.
Consider switching to showers when traveling and resign yourself to the fact that kids may look a little “rough around the edges” without their same bath routines.
(tips shared by patients for coping with their ichthyosis)
EXFOLIATION
Exfoliation is the act of removing the outer scales from the skin. For those individuals with hyperkeratotic ichthyosis (i.e. thickened skin) exfoliation can make you more comfortable doing day to day activities. And those with CIE, harlequin, or epidermolytic ichthyosis will find exfoliation most beneficial. However, exfoliation, while removing the scale, often brings out the underlying erythema or redness. So you may trade the scale for the redness. This is not an issue if the scale is very bothersome, but it is important to keep in mind if you do not want to be red. In other cases, such as Netherton syndrome, the skin barrier is impaired, and the skin should not be further exfoliated. Exfoliation will only lead to increased sensitivity and irritation in this case.
Tips for Exfoliation:
After soaking in a tub for an hour or longer, use a wash cloth, pumice stone or loofah brush to gently remove the outer layer.
After bathing, try using glycerin mixed with petroleum jelly or cream/lotion containing ammonium lactate 10% or 12% (Amylac, Lacsoft), glycolic acid (Glyco-A 6% or 12%), salicylic acid (Salicylix 6% or 12%), paraffin (Cetraben, Aquasoft), urea (Eucerin 10%, Revitaderm 20%), Mixed preparation e.g Moisturex to help soften and exfoliate the outer layer.
For those with thick scale under their eyes,topical tazarotene (Tazret) or tretinoin cream (Retino-A 0.025% or 0.05%) can loosen the scales and allow their eyelids to close more fully.
(Ref: Dr. John Browning, Chief of Dermatology at Children’s Hospital of San Antonio)
** If the skin is sensitive, or has some areas of open skin, some of the above mentioned products will sting. Consult with your doctor before using any of these products on an infant or child.
Use baking soda and make a paste which would be considered a mild abrasive to aid in loosening the dead skin.
Add Vaseline Bath Beads or apple cider vinegar to the bath water to help in softening up the skin before exfoliating.
You can try sugar scrub, sea salt or rock salt scrub which are available in body shops.
Moisturizing soaps likedove, olay, nivea, pears etc. can be used to scrub the loose skin off gently with palms or a wash cloth. Soaps rob skin of essential oils, so should be used occasionally. 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. If your child is sensitive to soaps, try baking soda or oatmeal bath additives which you will get in grocery store or drugstore. If your baby has raw areas on it’s skin, put petroleum jelly on that area before bath. This will reduce the pain when comes in contact with the bath water. After shower, pat dry the body with towel to keep the skin moist. Immediately apply cream or lotion mixed with glycerinto trap that moisture while you are still in bathroom. How often should you give your child a bath depends on the condition of the skin and the weather. If you live in a climate with dry, cold weather or too hot, you may have to give your child more frequent baths. Ichthyosis becomes worse in dry season. Steam of comfortably warm shower helps to clean skin & remove toxins from the body.
Let a loofah sponge soak in water for a few days before using, they will eventually soften up.
When exfoliating areas such as knees, elbows and feet, if it starts to hurt, stop, and continue the next day.
The salty ocean does wonders for the skin, and works as a natural exfoliant.
Hydrotherapy, whereby the water and air is circulated throughout the tub, may also be a beneficial way to soften and exfoliate skin.
(tips shared by patients for coping with their ichthyosis)
Dry & itchy scaly scalp is a common problem for ichthyosis affected people. If the scalp care isn’t done properly, it will be the suitable place for rapid growth of microorganisms.
The recommended way to treat scalp scales is to coat the scalp with your favorite lotion or oil to soften the scale, cover with a shower cap or plastic wrap and go to sleep; shampoo in the morning and brush or comb scales out after shampooing. Use a fine-toothed comb to pull up scales. A dog’s flea and tick comb is very fine, or use one made to remove head lice nits.
For children – wrap head in a bandana instead of a shower cap, or cover shower cap with a soft hat that ties under the chin to keep it from sliding around.
Products that may work are Zinc pyrithione, ketoconazole, salicylic acid, tar containing shampoos which are commonly used to remove scalp scale. Consult with your dermatologist before trying them.
Nizoral Shampoo with 2% ketonazole.
T-Sal Extra Strength Shampoo, by Neutrogena
Pantene Anti-dandruff Shampoo
Selsun Blue Shampoo
Seborin Anti-dandruff Shampoo
Mineral oil
** If the scalp is sensitive, or has some areas of open skin, some of these products will sting. Consult with your doctor before using any of these products on an infant or child.
