Meet Arienne
Newborn Story
Arienne was born in the same local hospital in Lugano, Switzerland as her two older siblings. We always joked that she had to do something extra special to secure enough attention from the parents of three children, but I have to say that we really weren’t expecting something as rare as EI.
When Arienne was born, it wasn’t immediately obvious from a clinical perspective that there was anything different about her. However she was extremely red, her hands and feet looked purple, she had a small wound on her head that didn’t make any sense, and she cried like the world was causing her pain. I have distinct memories of feeling very confused about the fact that somehow I instinctively knew that the wrist band they had put on her wrist was hurting her, even though her skin was intact at the time. Arienne was delivered full-term (5 days late) by vaginal delivery, and she was a very healthy 3.8kg, with a perfect Apgar score. No one had a clue that she had EI.
It was during her first night that I found raw wounds behind her knees and then quickly found the classic EI newborn blistering on her buttocks. More importantly, she howled in pain every time she peed, seemed constantly agitated, and everything I’d learned about how to soothe my other two children just wasn’t helping Arienne at all.
At first the Paediatrician dismissed my concerns and told me that she was born late and that this sometimes caused the skin to peel. This was our third child, all were born well after my due date, and I knew that what the Dr was saying was not accurate. The next 12 hours changed everything for us. Thankfully one of the midwives at the hospital knew about ichthyosis, because she has Ichthyosis Vulgaris herself, and she recommended that the team seek specialist support from the Children’s Hospital in Zurich. We will forever be grateful to her for becoming a midwife, and for advocating for us, because I know that she saved Arienne a whole lot of pain by getting her the attention she needed very quickly.
Arienne is 5 years old and is affected with KRT10 Epidermolytic Ichthyosis without PPK
Arienne was transferred by ambulance to the Children’s Hospital in Zurich, but I was not permitted to go with her during the night. Zurich thankfully sent a specialist team to come and prepare Arienne for her 3 hour ambulance ride. I cannot tell you how hard it was to be separated from Arienne at this time, however the Zurich team were kind enough to call me to let me know that she had arrived safely. This allowed me to get some sleep before travelling up myself the next morning. I was told to expect that she would be in the NICU for a month, and that I should make plans to accommodate this. I felt really lost at this news because I knew that I would have to be separated from my husband and other two children, who were only 2 and 3 years old at the time.
Thankfully, when I arrived in Zurich, the atmosphere around Arienne was much more relaxed. The team already had a care plan in place, and when I was reunited with Arienne she seemed very peaceful. She was being given morphine for pain relief at the time. The next few days consisted of me learning how to care for her, which included skin care, wound care, and learning how to bath her. There were some ups and downs in her NICU care, and sometimes the team tried a new approach that didn’t work so well, and some of the nurses definitely had more confidence than others. However, at the time, I didn’t really understand that there was so little knowledge of EI, so I thought that the nurses knew what they were doing, when in fact they were really just following instructions to the best of their abilities. This meant that sometimes, I would instinctively NOT want to do something, but the nurses had to insist that we followed the plan, even when it seemed clear to me that the particular direction was making Arienne worse. As the days passed, the clinical team started to recognise that my instincts were sound, and that I was independently managing Arienne’s care routine. The initial wounds which had been caused during her first few days had started to heal, and after 3 days we took her off morphine and found she was able to cope. By Day 6 we were discharged home, and it was such a relief to get out of the hospital and back into my own space to start to figure out what would eventually become our new normal.
This was our initiation into the world of rare skin diseases, and the beginnings of my understanding that I would become the expert, and that I was really the only one who could figure out how to help our daughter. After 5 weeks of diligently following the hospital care plan that took around 5 hours to complete each day, I decided that there had to be a different way. What I was doing seemed to take an extraordinary amount of time, and yet it still felt like we were drowning. Her skin still blistered relentlessly, and nothing I was doing seemed to make any sense anymore. So I decided to try and find some other people to talk to about it, and see if they could help me to understand what they were doing and why. Thanks to the UK Ichthyosis Support Group Charity and through the power of social media, I was able to find other parents of affected children, and adults with EI who were kind enough to share their own experiences and offered me some excellent advice. Together we started an EI support group, and what started with 5 kind families, has now grown to over 160 families from 30 different countries around the world. If you are reading this and would like to join our private Facebook group, please follow this link to connect with us.
Work, Childcare, and School?
Before having Arienne, I worked as the Head of Radiotherapy in a nearby Hospital, where I worked typical hospital hours. After many attempts to find someone or somewhere that could meet Arienne’s care needs in order for me to return to work, Tom and I decided that it really would be better and more financially viable for me to stay at home with Arienne. I know that some people successfully send their babies with EI to daycare, but for us, it just didn’t seem like the right thing to do, and I have had no regrets about the decisions that we made. We have been extremely lucky because Switzerland recognises the severity of a condition like EI, and we are offered some disability support for her care. This support has been invaluable, and has allowed us the financial stability that we needed.
Arienne is now 5 years old, and has just completed Pre-school, which she started at the age of 3 years. We are lucky that she is very verbal and able to communicate many of her needs, and she has been able to do this since the age of 2 and a half years of age. If it weren’t for this, and the fact that she is a sibling, I think that we would have waited another year to send her to Pre-School.
Care Priorities
Arienne is a truly super kid, and thanks to the EI Cure Project, and all our work in her school to raise awareness about rare diseases, everyone knows about her skin, and just accepts her the way she is.
As parents, Tom and I have always chosen to prioritise Arienne’s mobility with regards to her skincare routine. This means that we work really hard to keep the thick skin under control, and that we do our very best to ensure her skin is as healthy and wound-free as it can possibly be.
In the images below you will see many pictures where Arienne looks like any other happy and healthy child. In part, this is because we spend so many hours each day caring for her skin. You will notice that Arienne spent a great deal of time naked and playing at home when she was a baby, and that’s simply because this was one of the best ways to reduce the redness and blistering associated with wearing diapers, and seams, and all other things causing trauma to her skin. Arienne’s first summer was also exceptionally hot, with a high humidity index and temperatures exceeding 40 degrees celsius. It was probably the most challenging time we have ever encountered with regards to blistering and heat trauma wounds, but the end result was lots of naked-time at home, and any trips out were limited to the cool of the morning. It was hard to see all our friends and family out enjoying the summer in the afternoons and evenings, but it was just something that we had to do to ensure that Arienne didn’t suffer so much. The following year and beyond, her skin was much more robust, and we found that she could sweat well in certain areas provided we kept the thick skin under control, and so we were able to do much more. In short, that first summer of feeling house-bound was just a phase, and it worked for everyone’s benefit in the end.
Happy to be home
Simple cotton sleep suits and a soft surface to sleep on are a good combo
Post-bath naked time is very helpful for wound healing in the diaper zone
Bathing flat in a baby bath tub to ensure scalp and neck get the benefit of bathing
Lots of naked time is good in the first year
Padded high chair (Straps removed) reduced friction wounds on hips, bottom, and thighs
Legs always covered with thin layer of clothing to prevent book corners causing wounds
Diaper-free onesie time during hot summers
Portable bath tub (Brand: Gorilla tub) for use in the shower when on holiday
Flying high on her 3rd Birthday!
Daily challenges with EI Skin in the Early Years
The challenge with EI skin however, is that you can go from everything feeling and looking great, to blisters and skin infections within what feels like the blink of an eye. Some of the images below are examples of just how much things can change, but they also show how important it is to learn how to recognise problems, and to find the right skincare routine for your child. You can find more advice on these more challenging aspects of EI skin on our EI Resources page. It is important to understand that the images in the following gallery show changes in the skin that happened in the first year of Arienne’s life. Some of these problems occurred because I didn’t know what I was doing yet, or because Arienne got bitten by mosquitoes and had severe local reactions, and some of them show either bacterial or fungal infections. Whilst all of these things do happen with some frequency for EI skin, if you follow the advice in the Newborn and Infant Care Recommendations found on the EI Resources page, you will largely avoid many of the problems that we experienced for Arienne, and you should be able to reduce the risk of major wounds and infections from occurring too frequently. The images at the beginning and the end of the gallery show Arienne with her skin looking healthy. As Arienne grew, and I learned how to care for her, she had more and more good days, and many less days consumed by pain and wound care.
Post-bath naked time was a great time for 'Tummy time' on a plastic backed sheet laid on the soft bed
Classic EI water-blisters caused by finger-point pressure
Early newborn inflamed skin
Early scale development around 3 to 4 months shown on inner arm
Blisters caused by normal ankle lift for a diaper change, we changed to lie her onto a prepared diaper instead
Early scale development around 3 to 4 months shown on knee and leg
Typical scale and redness on thigh due to heat
Skin trauma due to heat and friction caused by being held by mum on a humid day
Scratch wounds following local reaction to mosquito bites on leg
Fungal infection on hip (Red bumps, skin looks shiny), treated with Imazol and 7-day course of oral Daktarin gel
Skin loss following heat and friction wound from being held on a humid day. Skin is now healing well, 1 day post-trauma
Frequent blisters, pus, and yellow crust around wounds denotes bacterial infection requiring treatment
Typical streptococcus infection denoted by deep red colour, excessive exudate, odour, and fast increase in wound surface area
Healing skin following wound debridement and treatment of skin infection with oral antibiotics
Appearance of wound following debridement and treatment for an extensive local reaction to a mosquito bite
Wounds caused by exfoliation attempts when skin was still too fragile
Skin dryness and flaking indicates need for a change in care routine. Addition of thicker emollients and use of wet-wrapping technique with Skinnies WEB resolved this
Happy baby once we got the care routine figured out!
Excess dryness and flaking lead to cracks and fissures in the flexures and folds. Additional emollients and wet-wrapping technique with Skinnies WEB resolved this
How does EI skin change over time?
When Arienne was a baby, her skin would blister a great deal with seemingly very normal every day activities. This meant that going in the car was traumatic, sitting in a stroller was traumatic, and even being held on a hot day led to traumatic wounds. The early months felt really impossible, and we all struggled to find our new normal, and yet somehow we did find our way through, and Arienne is a really happy, cheeky, and loving little girl.
The first year of life with EI is really the hardest from a physical care perspective, but we found that things started to get easier once Arienne’s skin started to show scale and got more robust, and she also learned to move and walk independently, which meant that she could change her position according to her own comfort levels, so the milestones of cruising and walking were really significant for our family.
When Arienne was a baby we were so anxious that she would never be able to crawl and walk without causing trauma to her skin. In reality, Arienne hit all her milestones at the same time as her older siblings, but the way she got there was slightly different. For example, she went from quickly from a bum-shuffle style crawl to an up on hands and feet bear-crawl, and then started to cruise up on both feet in a much shorter timescale than her siblings. She did experience a lot of redness on her legs and bottom from the bum-shuffle crawl phase, but as long as her skin was covered by a layer of cotton, we were relieved to see that the redness rarely turned to blisters if we applied some cornstarch or baby powder to the red areas after her evening bath.
The first 2 years of life with EI are hard from a care-giver perspective because it is mostly filled with wounds, infections, lengthy care routines, and there are a lot of unknowns about how the skin will change. Once your child is past the worst of the falling over phase of learning to walk and run, the anxiety of wounds and accidents subsides a bit because you learn that the wounds will heal very quickly (One of the bonuses of EI), and the typical hyperkeratosis starts to show it’s true nature. By 2 years, you understand your child’s EI skin needs, and then you can start to embrace your new normal.
By the age of 3 years, EI skin care no longer dominated my thoughts, and we started to find our rhythm again as a whole family. I remember our dermatologist telling us that we wouldn’t know what we were dealing with until Arienne was 2 years old, and feeling like that was an impossibly long time to wait to know what our child’s skin would be like. Hopefully, the information provided here can give you some idea of what to expect, and how to manage it. When I was a new parent with a baby with EI, I really wanted to know these details, and hopefully this website will help new parents to understand the natural progression of EI over time.
The other most significant milestone for a baby with EI, is getting out of diapers, particularly if you live in a hot and humid location like us. Diapers were one of the biggest causes of pain for Arienne during the summer months. She started to get awful wounds on her hips when she started to move more because the diaper tabs would cause lots of friction and skin trauma. We found that Pampers Pull-up pants were much more forgiving, with a soft and flexible side, instead of the usual diaper tab. In general though, during the summer, I decided to forgo diapers and the endless wounds that they caused in preference for doing lots of laundry. I bought a load of basic cotton jumpsuits, and Arienne lived in these during the summer. We went through 3 or 4 of these each day, because she would pee in them, but this felt like a good solution to the chronic hip wounds during the hot and humid summer months.
Arienne’s Care Routine
Between the ages of 6 weeks and 4 years, Arienne’s care routine consisted of twice daily baths followed by a full emollient regime. Our early emollient regime consisted of Dexeryl, applied after bathing, and after every diaper change, which meant we were applying emollient around 4 times every day. Around 4 months, the fragile newborn skin gave way to increasingly scaly skin, and we started to trial different emollients. We met by chance with another dermatologist from a big german research institution, and she advised us that the only emollient ingredient that is essential for EI are ceramides. She kindly gave us lots of different ones to try, and they all had a slightly different response from the skin, including colour absorption. The ceramide lotion we would eventually come to know and recommend the world over, is Aveeno Eczema Therapy, but I’m told that Cerave is a good second if this is more accessible to you.
Bathing
From around 12 months, in addition to emollients, we also started to use a keratolytic cream containing 12% urea to help to manage the thick scale on all joints and flexures. From around 15 months, we started to use baking soda in the bath, and the length of time that Arienne spent in the bath each time had increased to 30-45 minutes at a time. The addition of baking soda to the bath was one of our ‘Game-changers’ and I really wished that I’d started using it sooner. It works by changing the pH and softening the water so that the skin can more easily absorb it, and this means that skin cells hydrate and lift more easily. In turn, this means that the scale associate with EI which are essentially dead skin cells, will shed more easily from the skin surface. Baking soda baths effectively help to hydrate the skin AND allow the skin to shed more normally. As a guide, we use 180g of baking soda in a full European bath tub, but it’s best to test the pH (The aim is pH 7.9) because water hardness varies.
You can start to use baking soda in the bath once you child has developed extensive scale (And has therefore moved past the extensive baby blistering-skin phase), and as soon as you child is old enough to stay in the bath for at least 20 minutes to ensure the skin has had sufficient time to absorb water. Baths that are shorter than 20 minutes typically only cleanse the skin, but don’t allow for the hydration that balneotherapy aims to emulate. If you find that bathing seems to ‘Dry-out’ the skin, it’s really because you’re not doing it for long enough, or because there is bleach or chlorine in your water.
Exfoliation
After bathing, from the age of around 9 months, I would use a cellulose sponge to exfoliate the white and fluffy looking skin on the joints and flexures. From 3 years I also started to use a curette tool to work on any extra thick areas such as the ankles, knees, wrists, and elbows. I can highly recommend this tool because it makes light work of very difficult areas. It is a surgical wound debridement tool, which sounds dramatic, but in reality it’s a small curved blade, that allows you to gently scrape away the thickened ridges in order to relieve areas where the thick skin might otherwise harbour bacteria and limit joint mobility.
Bathing and exfoliation typically requires 1 hour per care episode for Arienne; 30-45 minutes for a bath that is long enough to ensure dead skin becomes inflated and easy to remove (Too long makes the skin too hydrated and sensitive, so there is a balancing act to this), followed by 15 minutes of exfoliation with a cellulose sponge and curette ‘debridement’. I find that 15 minutes is the most that Arienne can tolerate in one sitting, which means that I alternate between her top half and her bottom half from care episode to care episode.
Exfoliation tools
Moisturisers
The skincare basics
The remainder of the care episode consists of the strategic application of many different keratolytics, emollients, and humectants to ensure that Arienne’s skin will stay comfortable, flexible, and flake-free for as long as possible. The first stage is the application of keratolytics to the thick skin found on the flexures and folds. Before the age of 2 years it is necessary to get a prescription for urea creams from a paediatrician, and it is really between the age of 9 months and 24 months that the true long-term hyperkeratosis of EI starts to develop. Over this period, EI skincare becomes less about managing wounds and more about trying to control the thick skin in order to prevent infections from developing (The thick skin gives bacteria and fungus more surface area to grow on), but also to ensure that physical development and mobility is not limited by hardened skin on the flexures and folds.
Between 12 months and 2 and a half years (30 months), we relied on a 12% urea cream called Widmer Carbamid cream, and then from 2 and a half years we began using Ureadin Ultra 40, which is a 40% urea gel on all the thick skin on the flexures and folds. I learned about how the different strengths of urea affect the rate of hyperkeratosis from studies published about urea being used for many different skin disorders including ichthyosis and psoriasis. I learned that 10% urea and below basically helps to soften the skin by attracting water. If you use a strength of urea which is greater than 10%, you will find that it helps to soften the thick skin, but it also reduces the rate of hyperkeratosis. This means that the higher the strength of urea, the more it can slow down the rate of skin growth. In order to find our new urea product, we tried many different ones, with permission from our dermatologist. Amongst the 5 or 6 different brands with 25-40% urea, we tried Eucerin 30% urea, but found it stung and turned the thick skin orange, we also tried asking our pharmacist to mix 40% urea with Dexeryl emollient, but it didn’t seem to have the desired effect. In the end we found a 40% gel product (Ureadin Ultra 40), which is designed to treat calluses, and found that it could be applied easily to the skin, soaked in well, and had a nice scent to it, but most importantly it actually helped reduce the thickness of the skin, helped to prevent cracks and fissures from forming, and helped to keep the skin tone more balanced (Thick EI skin looks darker than normal skin tone).
The first step to moisturising Arienne’s skin post-bath and exfoliation, is to apply Ureadin Ultra 40% urea gel to all the flexures and folds. After this, we apply a mixture of emollients with vegetable glycerin all over her body. Each of the ingredients in this mixture are carefully chosen for a different purpose. We use Aveeno Eczema Therapy because it contains oats which help to nourish the skin biome, and ceramides which ichthyosis skin is proven to be lacking. Aveeno Eczema Therapy is therefore what we consider to be a ‘Treatment’ to the skin. However, for EI skin, Aveeno Eczema Therapy it is not moisturising enough, so we add some other things to help.
What we add will vary depending on whether Arienne has just had a bath or not, and will also vary depending on the season and how much humidity is in the air on average. For example, during Winter, we need thicker creams which contain paraffin, but in summer, those types of thick creams could cause excess heat which leads to blistering. In addition, vegetable glycerin should only be used after bathing, so we don’t use it if Arienne needs moisturisers in-between bathing sessions. This is because glycerin is a humectant, which means it draws water to it. If we apply glycerin to surface skin cells that are hydrated with water from a bath, the glycerin will help to keep that water in the skin for longer. If however, we apply glycerin to surface skin cells that are dry, the glycerin will draw water out of the deeper layers of skin. This is NOT what we want to do, because it means that we are ultimately making the deep layers of skin more dehydrated, and this can lead to deep cracks and fissures if we do this in the long-term. In summary, we only use vegetable glycerin after bathing, and here I will summarise our moisturising strategy:
Typical Post-bath Moisturisers: Ureadin Isdin 40% to all joints and flexures, followed by mixture of Aveeno Eczema Therapy (5), Dexeryl (2), Lipikar AP+M (2), and glycerin (4)
Typical In-between Moisturisers: Mixture of Aveeno Eczema Therapy (5), Dexeryl (2), and Lipikar AP+M (2)
PLEASE NOTE: the numbers shown in brackets demonstrate the number of pumps used for each moisturiser added to my mixture. I keep all of my moisturisers in separate pump dispenser bottles, because I find it easier to mix into my hands as I go. I keep the ratio of emollients to glycerin at approximately 70:30 because this is documented as safe in academic literature for ichthyosis. It is important to use emollients because they contain essential ceramides and lipids which help to treat the impaired skin barrier. Many people report only using vegetable glycerin on their skin, whilst this may work in the short term, it will not nourish the skin biome and skin barrier in the same way that an emollient can. I therefore use emollients to ‘Treat’ the skin barrier, and use vegetable glycerin to ‘Boost’ the longevity of hydration post-bath. Hopefully you can see from the pictures of Arienne’s skin in the gallery below, that this works very well. Arienne has moderate to severe EI, but most people see her skin and think that she is a mild case. This is not true, but the healthy condition and appearance of her skin is really because we care so meticulously for her skin every day.
Scalp Care
Like most with EI, Arienne get scales on her scalp. Provided we keep on top of this, it is not too bad. I know it’s time to do our scalp care routine again when I start to see flakes, and she starts to scratch at her head in an absent-minded way, but as a general rule, we do this once every 2 weeks.
We have two different approaches to a full scalp treatment depending on how extensive the scales are:
Before bed, brush hair and cover scalp in a thick layer of Aveeno Eczema Therapy. The head should be wrapped with a hair towel or clean muslin cloth to wet-wrap the head, and to protect bedding from being soiled. This will help to soften the scales overnight, and prepare for further treatment during the morning bath routine. In the morning, soak the head in the morning bath, and gently massage the scalp with a silicon exfoliator brush (Pink and Blue tools shown in the ‘Exfoliation tools’ picture above). After this, use a comb or head-lice comb to brush the hair from the scalp. This should help to remove flakes and scales.
Plan for a longer morning bath than usual. Soak the scalp well, and then apply an oil of your choice all over the scalp. Our product of choice is Anti-Dry Bath Oil (Which contains almond oil), or Now Foods Argan Oil. Leave the oil on the scalp for a good amount of time, usually 15-20 minutes works well. Rinse off the excess, gently massage the scalp, and then comb slowly through the hair as described in 1).
After completing one or both of the above approaches, we wash the hair and scalp with Lubex Ichthyol Shampoo, then rough dry the scalp thoroughly with a towel.
If the scale on the scalp is particularly troublesome, it may be necessary to combine both 1) and 2) for 3 or more consecutive days to help clear the scalp, before going back to the maintenance regime of every 1 or 2 weeks for scalp care.
Skinnies WEB garments
Wound Care Tools
Wet-wrapping with Skinnies WEB garments
Despite all the care taken on our moisturising strategy, we would find that Arienne’s skin would soon dry out after a few hours. To remedy this, we use special breathable and seam-free clothing to effectively wet-wrap her skin. From the time that Arienne was able to stand independently, we started to rely on Skinnies WEB clothing to help keep Arienne’s skin hydrated, flexible, comfortable, and flake-free for longer. Skinnies WEB were also really helpful in protecting Arienne’s skin from superficial wounds, for example, a minor trip or fall without skinnies would cause the skin to rip and tear, whereas skinnies often prevent the skin from being damaged in the first place. I therefore highly recommend Skinnies WEB Garments as one of our “Essentials” for happy EI skin.
Skinnies WEB are essential for the fact that they are designed to hold wound dressings in place. This is important because EI skin cannot tolerate any kind of adhesive dressing. This brings us to another very important topic of Wound Care, and you can see all the tools in our wound care kit in the above picture.
Recognising and Managing Wounds
How we manage wounds and blisters really depends on what caused them, and in our experience there are 4 different causes: 1) Trip or Fall, 2) Allergy or Local reaction, 3) Friction/Heat, 4) Infection (Bacterial or Fungal). Some aspects of wound care are common for all these causes, but there are also distinct differences in how we manage them. An in-depth description for how we manage these different types of wound can be found in the Newborn and Infant Care Recommendations on our EI Resources page, but I will briefly describe what we do for each type of wound below.
1) Trip or Fall wound
2) Blisters caused by Allergy or Local Reaction
3) Blisters and Erythema caused by Friction/Heat
4a) Pus and yellow crusting denotes Bacterial skin infection
4b) Small red dots and shiny red skin denotes Fungal skin infection
Trip or Fall wound: It is typical for the skin to tear and reveal a superficial raw wound with a skin flap. Clean the wound with saline or wound wash, and remove the skin flap as cleanly as possible. Mix a little zinc paste with antibiotic ointment to reduce the risk of infection, and then cut a 2-ply thickness of sterile gauze to cover the wound. If the wound is covered by skinnies, the gauze can go on the wound, then will be held in place by skinnies. If the wound is NOT covered by skinnies, the gauze can be held in place by the ‘Self-adhesive soft blue bandage’ shown in the green box.
Blisters caused by Allergy or Local Reaction: Water-filled blisters can appear directly in response to something which was in-contact with the skin (Bug bite) and caused a local reaction, or they can appear all over the body as a systemic reaction (Drug reaction or Food Allergy). Blisters in this case can start quite small, but may increase in size over time, and in the case of a systemic reaction, there can be many small blisters all over the body.
In either case, we treat the reaction itself AND we must manage the wounds. To treat the reaction, we give prescription medicines which include antihistamines (for 3 consecutive days or until the reaction starts to resolve) and depending on the severity of the reaction, we may also give a single high-dose of an oral corticosteroid. If the reaction is a systemic one, likely caused by a drug reaction for example, it may also be necessary to give a short course of an oral corticosteroid to reduce more widespread inflammation.
The blisters should start to dry up and look and feel less inflamed with the help of the medicines, however blisters can be painful because of the pressure when full of fluid, so we always lance any large blisters. To lance a blister, it’s best to make a small incision with either a sterile needle or scissors so that any excess exudate will drain according to gravity following your initial treatment. Blisters caused by an allergy tend to continue to exude fluid for a day or two after the initial reaction. If the wound stays very wet, it’s best to remove the roof of the blister (top skin flap) to ensure that the wound can be properly cleaned and that bacteria is not trapped inside the wound.
To lance the blister, make a small incision, compress with sterile gauze soaked in wound-wash or saline, apply cornstarch or baby powder to the intact roof to help dry the wound, then apply a mixture of antibiotic ointment with zinc to help prevent infection and to help dry the wound up further. From this point, we manage the wound as described for a trip or fall.
Blisters caused by Friction/Heat: When the cause of blistering is friction or heat, the skin in the affected area often looks very red, inflamed, and angry, with some fluid build-up beneath the surface. The skin looks very thin, with clear fluid just below the surface, and the affected area can be quite large. It looks completely different to a typical small, round blister. In the first instance, it’s important to remove the source of the trauma (clothing seam, shoes, car seat, even being held can cause trauma), and then try to prevent further blistering by applying cornstarch or baby powder very gently to all the skin that looks inflamed. For any blisters, lance them as described above, then apply cornstarch or baby powder to help absorb excess fluid. Once dried further, it can be helpful to apply a mixture of antibiotic ointment mixed with zinc paste to prevent infection, and help dry the wounds up further. Typically, I wouldn’t apply dressings to wounds like this, because the dressing will just increase the heat to the skin surface, and the inflammation needs to go down.
It can also be helpful to ensure any usual baths which follow an episode of extensive redness from friction, are a little more cool, and are kept short. The bath will ensure wounds are clean, but you don’t want to add more fluid to the inflammatory response with the usual long soak, so keep a post-trauma bath to a maximum of 15 minutes. If there are any raw areas, these will sting upon entry to the bath. To prevent this, it can be helpful to apply a thin layer of vaseline or other ointment over the raw wound, this will protect the wound from the initial entry into the bath, and it will gradually rinse away to ensure the wound gets clean without the tears.
Blisters and Wounds caused by Infection: A blister caused by a BACTERIAL skin infection typically contains cloudy pus, or it might have a yellow crust around it. If there is a single wound like this, you can usually treat it topically with antibiotic ointment. It’s really important to de-bride the wound and clean it out as much as possible before sealing it with antibiotic ointment.
Different types of bath can also be helpful to draw out infections. Examples include: salt, apple cider vinegar, bleach, or potassium permanganate. In the first instance however, I typically use our usual antimicrobial agent in the bath (Octenisan wash), soak for 15 minutes (Shorter than usual 30-45 minutes to prevent the wound from being over-hydrated and hyper-fragile), and then set about removing all the dead and infected tissue from the wound. Once the wound is free from dead tissue, soak sterile gauze in wound wash, and gently apply to the wound. Let it dry, then help it to dry out properly by gently applying cornstarch or baby powder around the edges of the wound. At this time, I go about the usual skin care routine, leaving the wound to dry out at the same time. Once finished, the wound should be looking more dry. I then apply an antimicrobial gauze dressing (Lalugen Plus) to the wound (Because I know that the wound will exude and needs to be helped to drain with the right dressing), apply antimicrobial cream (Lalugen Plus) to treat the infection, cover with 2 ply sterile gauze, and then hold everything in place with Skinnies WEB or Self-adhesive soft foam bandage. I often take a picture of wounds like this, as a point of reference to see if it has improved next time I look at it, which would typically be after another 10 to 12 hours.
If the wound looks worse when I next check it (It looks more red, more exudate, more pus, and it’s grown in size), then I know that the infection is not under control and I need to change my management. At this stage, I have 2 choices: 1) Visit the Doctor’s office for a swab and a prescription for oral antibiotics; or 2) I can use more aggressive home remedies such as an apple cider vinegar bath. In general though, I will persist with treating at home for a maximum of 2 days, but if the wound continues to accelerate, I prefer to treat it quickly with oral antibiotics, but insist a skin swab is done to ensure I know what the source of the infection was (E.g. Streptococcus or Staph. Aureus). Streptococcus can be particularly troublesome if not treated quickly and aggressively, so it’s important to advocate for a swab BEFORE starting antibiotics to ensure the correct antibiotic is given.
Blisters and wounds associated with a systemic skin infection are usually quite dramatic. It can seem as though a local infection suddenly turned into a wild fire, and blisters pop up everywhere and without any apparent cause. In our experience, systemic skin infections aren’t always associated with a fever, and the only sign is the fact that blisters are appearing everywhere in a very short time-period. If this happens, we know an infection is present and we need medical help quickly.
It is typical to see rapid improvement in the appearance and feel of wounds associated with a bacterial infection once the right antibiotics are given. If a clear improvement is not observed after the first 4 doses of oral antibiotics have been given (i.e. reduced blistering, less exudate, less redness), and a swab to check which type of bacteria is causing the infection was NOT already performed, it is best to contact the prescribing physician to enquire about taking a swab because the given antibiotic is not working. This means that either the wrong antibiotic has been given for the type of bacteria present, OR the infection has a different cause (Which might be a fungal). Only an accurate swab, ideally taken from the centre of more than one wound bed (Not exudate), will determine which course of treatment is needed.
A FUNGAL skin infection looks quite different, and these usually only happen in skin flexures or folds that are naturally more moist, and most often occur within the diaper zone. The skin becomes red and shiny, and may contain small red dots, but blisters are generally not common for a fungal skin infection. The skin is also very itchy. Use a cool gel pack to relieve intense itch, but medical treatment is needed. An apple-cider vinegar or oat bath can also be used to help rebalance the skin biome if you want to try a home remedy before medicating. For the medical approach, we use Imazol anti-fungal cream twice a day for 7 days, and if this does not start to improve things within the first few days of treatment, we also use Daktarin Oral gel for 7 days. If this does not work, a skin swab and fungiogram are needed to identify which fungus is in excess, and which anti-fungal it is susceptible to.
What does Arienne’s skin look like most of the time?
As you can see from the pictures of Arienne’s skin below, our care routine works very well for Arienne, and she is able to move and play as freely as her siblings as a result. The problem however, is that is all takes such a long time, and this in itself goes against our desire for Arienne to be as active and happy as her siblings. To do this care routine twice a day takes between 4 and 5 hours everyday, and over time this felt increasingly unsustainable and we also had to consider the needs of our older children and quality of life for our family. Spending all this time on skin care meant that there was so much less time in the day for us to spend together as a family, and that the kids all had so much less time for play.
Acitretin
When Arienne started Pre-School, it became much harder to fit all the hours of daily care around the school day, and around the needs of Arienne’s Brother and Sister. Arienne needed to get up at 6am each day in order to get to school for 8am, and we really started to struggle to fit in the second bath and emollient regime as well. Arienne was extremely tired after her school day, so trying to do a healthy dinner for everyone, help my older kids with their homework, support any after-school activities, AND fit in 2 hours of bath, scrubbing, and creams felt like an increasingly impossible task. Yet, we really didn’t want to compromise on Arienne’s comfort and quality-of-life from her skin. We tried cutting back on her skin care routine, but it just led to thick skin that felt out-of-control, in addition to the development of painful cracks and fissures on the joints and flexures. We tried using the weekends to catch-up with extra skin care due to the time lost on school days during the week, but this just led to lots of frustrations around extended care times, and not enough family fun.
In the end, we decided to try giving Arienne Acitretin, which is a medication that helps to slow down the rate of skin growth. There is often a lot of controversy about the use of Acitretin, and this is partly because early use of it for ichthyosis involved very high daily doses to normalise the skin. These high doses led to many unwanted side-effects including liver damage and significant joint problems. In some cases, Acitretin even led to changes in bone development, and so this medication is something we really had to consider carefully.
Thankfully, the use of Acitretin has been studied more extensively, and around the time that Arienne turned 3 years old, some new paediatric consensus recommendations for the use or retinoids in ichthyosis were published. These guidelines were written following an extensive review of all the literature on paediatric uses for Acitretin. The conclusions from a panel of ichthyosis experts were that Acitretin is safe provided that careful blood monitoring is undertaken, and that doses are kept low. The result is that Acitretin does not normalise the skin as seen with the high doses in the past, but instead it helps to reduce the rate of hyperkeratosis and therefore reduces the time needed for care every day.
It is also important to note that Arienne has KRT10 EI, and Acitretin has been shown to work very well for this specific genotype. Unfortunately, for KRT1 EI, Acitretin is often problematic, leading to more inflamation, fragility, and increased blistering, and should therefore be used with caution for anyone with a KRT1 genotype.
At the age of 4 years, 3 months after Arienne started Pre-school, we did a trial with Acitretin. Our dermatologist was very supportive and reassured us that she had very few concerns about Acitretin, and that to be safe, we would start on a very low dose to see how Arienne’s skin would respond.
I have to say that Acitretin was life-changing for Arienne and our whole family, and this benefit was achieved with very low doses. Even now she is no where near the full dose that would still be considered safe for her. We were able to drop the second care episode from Arienne’s daily routine, and now we only spend 2 to 2 and a half hours each day caring for her skin instead. When you are used to trying to fit 4 to 5 hours of care into every day, being able to drop this by 50% felt so incredibly liberating for our whole family. Acitretin gave us exactly what we needed in order to give us the right balance between time for essential care alongside our desire to maintain family bonds and the all-important time for play. Of course, we would ultimately like for Arienne not to need to spend 2 to 2 and a half hours each day on skincare, however, Acitretin has made all of our lives more manageable and Arienne is much happier and more active as a result.
Contact Details & Further Resources
If anyone would like to know more about the evidence-base behind Arienne’s skincare routine, you can find much more detail on our EI Resources page. If you would like to get in touch to talk about our skincare routine, please feel free to contact me on helenlill.eicureproject@gmail.com. It took me a very long time to figure out a good care routine for Arienne, and I’m very glad to help others to achieve the same thing. No matter what, EI care is tough and can feel relentless at times, but with the right approach we have found life can be full and joyful, and that the only limit to our days is that we must always factor into our plan the 2-3 hours we need to do the care routine.
