Newborn and Infant Care Recommendations for Epidermolytic Ichthyosis (EI)
Care recommendation Rationale
1 Do NOT use a humidified incubator [1,2,3,4]
EI skin is more prone to blistering and fragility in hot and humid conditions [1,2] In addition, EI skin is frequently affected by anhidrosis (Inability to sweat), so too much heat can cause overheating [3]. Signs of overheating include redness, scratching, and in the later stages of heat stress, sudden lethargy and losing consciousness If an incubator is required for other care considerations (E.g. premature delivery), humidity should not be used [4].
2 Dress in light and breathable fabrics such as cotton, bamboo, or muslin. Minimise contact of clothing seams with the skin, and remove all labels and tags. Dress in full bodysuit including coverings for hands and feet when possible. Consider turning clothes inside-out to reduce contact with seams [3,5,6]
EI newborn skin is extremely fragile and unstable, and any amount of heat and/or friction is likely to cause redness and/or blistering [3,5,6]. It is therefore imperative to ensure clothing allows the skin to breathe, and does not trap moisture in any way. Fabrics like polyester or fleece are to be avoided. Clothing should be checked for tags, labels, seams, zips, buttons, and anything else that may potentially rub against or be in direct contact with the skin. Anywhere that a seam is in contact with the skin may result in trauma, so a full bodysuit with coverings for hands/feet is best because it provides protection for the whole body, but also minimises the number of seams that could be in contact with the skin in comparison with those found in an outfit composed of multiple items of clothing. Clothing can be turned inside-out to ensure seams are not in contact with the skin, and any tags or labels should be removed.
3 Minimise skin trauma from handling [4,5,6]
EI skin in newborns and infants is extremely unstable and fragile and will easily tear and blister with usual handling [5,6]. Care must be taken to reduce skin trauma using the following techniques: 1) always place baby down onto a muslin square or light cotton blanket so that the cloth can be used to lift baby enough to slide whole palm underneath; 2) place palms on soles of feet to lift legs and bottom when changing a diaper, instead of pulling on ankles; 3) ensure clothing is easy to put on and ideally lower baby down onto open clothing and hold clothing for baby to push arms/legs into, rather then trying to move arms/legs into clothing. Where possible, avoid the use of gloves when handling, because these can cause friction and tear the skin [4].
The epidermis is unstable in EI skin, and detaches easily with even the slightest pressure, heat, or friction. This is most acute in a newborn, and over time thicker more classic ichthyosis scales or hyperkeratosis will develop. Whilst it is extremely tempting to bandage the skin and use dressings to protect it, these approaches typically result in more wounds developing because of the handling required to apply them and because the wrappings and dressings simply trap heat and moisture on the epidermis [1,4,7]. If a dressing must be used, ensure it is
Written by Helen Lill: eicureproject@gmail.com5 Minimise skin fragility due to excess moisture [3,4,5,9,10,11]
non-adhesive, and that it allows fluid to be drawn out of the wound (E.g. Mepilex transfer or Mepitel One), however if the wound continues to increase in size, this should be taken as a sign that the dressing is trapping heat and causing further trauma [1,4,8]. The best approach to managing wounds in EI, is simply to disinfect the denuded skin, allow it to air-dry as much as possible, and then seal the wound with an occlusive ointment (E.g. vaseline). It is common practice in the EI community to use antibiotic ointment mixed with a little zinc paste on these types of wounds (See “Care recommendation 20 and 21”).
Typical areas for wounds in EI newborns include the skin folds and other areas that are naturally moist such as groin, armpits, backs of knees [3]. As much as possible these areas need to be given time to dry out before applying any kind of moisturiser. Ointments should never be applied to these areas when the skin is damp, because this will make the skin soggy and more fragile [4,5]. After bathing it is good practice to use clean gauze or a clean muslin cloth to help these areas to dry up. Only once dry, should a breathable lotion be applied to these areas, such as Aveeno eczema therapy or Cerave baby [4,5,9,10,11] For the diaper zone, once the skin is dry, apply the same light lotion, wait for it to absorb, remove any excess, and then apply Bepanthen diaper salve to protect the skin from faeces and urine [4].
6 Maximise time spent without a diaper [1,4] The diaper zone is one of the toughest areas to treat for a newborn with EI, because it is such a moist area The skin in the diaper zone will heal and become less red very quickly if permitted an appropriate time to dry out every day. Ideally babies should be laid onto an absorbent pad to ensure urine is soaked up and does not irritate the skin [1,4]. When it is essential to use a diaper, follow details in “Care recommendation 5”
7 Minimise risk of infection [8,9,10,11]
Those with EI have an impaired skin barrier function, in addition to hyperkeratosis in some areas. These two factors can lead to colonisation of bacteria and fungus on the skin [8]. If not kept in check, colonisation can easily lead to infection, therefore it is important to use the following approaches to minimise the risk of infection: 1) ensure use of sterile technique as much as possible; 2) cleanse wounds with disinfectant/wound wash/saline; 3) seal wounds with occlusive ointment; 4) use antimicrobial solution in the bath [8,9,10,11].
8 Use non-occlusive creams/lotions as moisturisers [2,3,5,11,12,13,14]
Written by Helen Lill: eicureproject@gmail.comEI skin in the newborn is typically more fragile than other types of ichthyosis, and the acute care need at this stage is to minimise the occurrence of wounds. Occlusive ointments should never be used as a general moisturiser for EI skin despite being recommended for other types of ichthyosis because they: trap heat and moisture (which leads to increased blistering), cause friction when applied, and form a barrier at the surface of the skin which actually traps old skin at the surface which needs to slough off [11,12,13,14].
It is therefore recommended to use breathable and easy-to-apply lotions such as Dexeryl, Aveeno Eczema Therapy, or
Cerave Baby to moisturise the skin. These should be used multiple times each day as required in accordance with how dry the skin appears to be. As the baby gets older, it is common practice to mix the lotion with vegetable glycerin after bathing to reduce the need for such frequent applications, but in the newborn stage, it is typically not skin dryness that is the acute problem, this will develop more over time [2,3,5].
Ceramide lotions are proven to help to repair the skin barrier and moisturise the deep layers of the skin without occlusion [12,13,14]. Aveeno Eczema Therapy and Cerave Baby are well known and effective ceramide lotions commonly relied upon by the EI community They help to smoothe scale and reduce itch, and can improve the condition of the skin significantly within the first few days of use.
Occlusive ointments such as vaseline, 50:50, and Aquaphor have traditionally been used as general moisturisers for all types of ichthyosis [3,4,5,6] They have been found to increase the frequency of blistering in EI, and as such should not be used as general moisturisers [2,3,4,5,6,15]. However, they are very effective for protecting wounds, and should be used for this purpose only It is important to allow a wound to stop exuding before applying an occlusive ointment however, so as to ensure the wound does not become water-logged. If a wound appears to grow in size after an occlusive ointment has been applied, the wound should be cleaned and helped to dry out better before being sealed (See “Care recommendations 20 and 21”).
See “Care recommendation 5”. Bepanthen diaper care ointment is a barrier ointment that aids wound healing, provides a barrier from urine and faeces, whilst still allowing the skin to breathe As such, it is very helpful for EI skin both in the diaper zone, but also can be used to heal cracks and fissures that can appear anywhere else on the face or body with EI skin [4]. It is worth noting that other brands of barrier ointment are not equivalent to Bepanthen, so it is best not to substitute if possible For product info:
https://www.bepanthen.co.uk/bepanthen-range/baby-and-parent/bepanthen-nappy-care-ointm ent
EI skin is comparable to two co-existing conditions, there is both fragile/blistering skin, and dry/thick hyperkeratosis [3,4,5,8,11]. The same management cannot be applied to both types of skin, and areas of denuded skin can be immediately adjacent to thick skin. Fragile skin needs to dry out, whereas thick skin needs more moisture. It is therefore important to avoid application of moisturisers and lotions to wounds, allowing wounds time to dry out and heal, whilst ensuring other areas are moisturised as required [16,17]. It is common practice in the EI community to use zinc barrier paste mixed with antibiotic ointment (all wounds outside diaper
13 Use emollient cream on gauze to clean faeces from diaper area [4]
zone) or zinc barrier paste mixed with Bepanthen barrier ointment (all wounds inside diaper zone) to help wounds to dry up, and to seal/protect the edge of a wound from the surrounding skin that needs to be moisturised. If the edge of a wound is not dried appropriately, the wound will continue to grow [4].
Faeces, and in particular meconium, can be very tacky on the skin, and this can cause trauma which may result in blistering and denuded skin [4]. The use of emollient lotion on gauze will minimise friction when cleaning/wiping the skin on the bottom, and reduce the risk of additional skin trauma [4]
14 Remove inner seam from diapers [4,17]
It is common practice in the EI community to remove the inner seam from diapers in order to reduce the risk of skin trauma from the diaper [4]. It is also advisable to use the softest diapers available, and to consider using pull-ups instead of tab diapers once a baby is able to sit up and crawl independently [4] The sides of a pull-up are softer and cause less friction on the hips than a tab diaper. It is debatable whether an over-sized diaper is preferable to the correct size. The rationale for an oversized diaper is to minimise friction, however a correctly-fitting diaper may be preferable to an over-sized one simply because less of the skin is exposed to the additional moisture from a diaper. In practice, it is best to try to find the right balance between facilitating diaper-free time and the need to wear a diaper [17]. If enough diaper-free time is possible, the skin in the diaper zone is much less fragile, and consequently correctly-fitting diapers are possible.
15 Ensure morphine is used ONLY if wounds are extensive during the first few days, then aim to stop as soon as possible [6,8,18].
It is typical for EI babies to have significant wounds from handling in the first few days after being born. Raw skin is extremely painful, however once the skin is sealed correctly, the pain is reduced significantly If managed correctly, these acute wounds should heal very quickly, and therefore morphine should not be used routinely past the first week of life [6]. If wounds appear to linger beyond the first few days, this indicates a different approach to care is needed If the source of skin trauma is removed, EI skin will heal very quickly, so it is important to identify the cause promptly and not continue with analgesics without investigation (See “Care recommendations 3, 4, 5, and 6” above) [6,8,18].
16 Use paracetamol when pain-relief is appropriate [18]
EI wounds are painful, and whilst they do heal quickly, it is important that a baby with healing wounds is able to get adequate rest and also to try to minimise trauma during wound care. If a baby has many wounds it is OK to give paracetamol prior to bathing or performing the care routine [18]. It is recommended to try to give medications 20-30 minutes prior, or to give it to them at bedtime to help the baby to rest and recover without discomfort [18].
17 Identify local bacterial infections Wounds and blisters are a frequent occurrence in the early years with EI, and consequently
Written by Helen Lill: eicureproject@gmail.com EI Newborn and infant care recommendations Version 1 Nov 2022and treat promptly with topical antibiotic ointment [8,10,17,19-22]
the risk of local infection is high [8,10,17,19] Signs to look for include: 1) Redness and inflammation increasing around the wound; 2) Cloudy pus inside a single blister, or a collection of pus-filled spots; 3) yellow crust around a wound [20]. Provided there are no other signs of systemic skin infection (See “Care recommendation 18”), it should be possible to clear the infection using topical antibiotic ointment [20]. It is important to try to debride the wound, meaning to remove old or sloughing skin and neaten up the wound edge/ remove loose skin as much as possible so as to reduce the risk of the wound harbouring bacteria [21]. This can be done manually using sterile gauze soaked in saline or wound wash, wound edges can be assisted with small sterilised nail scissors or a curette tool, or the wound can be cleaned up using a debrisoft pad or debrisoft lolly [21]. Once the wound has been cleaned and sterilised, it can be treated with antibiotic ointment (E.g. Fucibet, Mupirocin, Neosporin, Bactroban, Triple Antibiotic ointment, etc), which should be used twice daily for up to 7 days [8,22]. It is also important to note that regular bathing helps prevent infection (See “Care recommendation 22”).
Systemic skin infections can occur as a result of a local infection spreading from a wound, or they can occur without the presence of a primary wound source [8,22]. EI skin has impaired barrier function, and may also be associated with excess scale [10,17] Both of these circumstances mean that bacterial colonisation can lead easily to systemic skin infection [10,17]. This can sometimes occur following an illness, when the immune system is under more stress than usual In babies it can be common during prolonged periods of teething When the infection comes from a primary wound source, it is typical for more small blisters to appear locally around the original wound, and then for frequent blisters to appear in any location on the body [8,19,20] Skin suddenly becomes much more fragile, and blisters appear frequently and seemingly without usual cause (Heat/friction/trauma) [20]. It is common for there to be no other indications of infection, such as fever or other typical symptoms of illness [20] Once a systemic skin infection takes hold, it can spread very quickly and the blistering can be intense and dramatic. It is sometimes possible to stop the spread of infection using salt water baths or bathing in potassium permanganate to draw out the infection [21,23], but if the blisters continue to happen frequently, and do not appear to dry out, it is necessary to give broad spectrum oral antibiotics [8,20]. It is also optimal to take a skin swab or scrape of an area of skin most affected by infection, in order to identify the bacterium and modify the type of antibiotic used if necessary [8,20] It is standard practice to start with a broad spectrum antibiotic whilst waiting for the results of the skin swab, then adjust the antibiotic if necessary. It is also common for those in the EI community to need a longer duration of antibiotic therapy than is typically used, for example, a 5/7-day prescription often results in the infection returning, whereas a 10/14-day prescription seems to be more effective. It is also important to
Written by Helen Lill: eicureproject@gmail.com19 Identify fungal skin infections and treat with antifungal creams and oral antifungal medicine [8,19-22]
note that regular bathing helps prevent infection (See “Care recommendation 22”).
An impaired skin barrier function also means that EI skin can be more prone to fungal skin infections, and this is more commonly seen in babies wearing diapers, particularly when they have folds of skin that may be moist [10,17,19,20]. The appearance of the skin with a fungal infection is different to a bacterial infection: the affected area looks red and shiny, and any wounds in the area have ragged edges. Alternatively little open spot wounds will appear within the red shiny region. Treatment includes a topical cream (E.g.Imazol) to be applied 2/3 times daily for 7/10 days, and if deemed a widespread infection, the treatment may also include an oral medication which comes as a gel to be given twice-daily by mouth (E.g. Daktarin) [19,20]. The groin, buttocks, and neck are the most commonly affected by fungal infections in babies. To help prevent this from happening or to help the antifungal treatment to work more quickly, it is good practice to allow plenty of diaper-free time, and also to try to help the neck to extend when napping (This can be done by lying the baby on a pillow, with the head extended over the edge on a comfortable safe surface) [20] It is important to note that regular bathing helps prevent infection (See “Care recommendation 22”).
20 Lance all fluid from blisters. If this cannot be achieved completely, it is better to remove the roof (de-roof) entirely and then treat the wound [4,24,25]
Water blisters can vary in size (A few millimetres up to many centimetres) but they can be large and are not self-limiting (Very similar to those seen in EB Simplex) [4,24]. Whilst it may be considered clinically controversial, within the EI community it is considered best practice to lance fluid from blisters or to de-roof the blister and then treat the wound [4,24,25]. The rationale for this practice is 1) to relieve pain from the pressure of fluid in the blister; 2) to reduce the risk of the blister becoming infected; and 3) to stop the wound from growing [4,24,25]. To lance a blister it is best to use a sterile needle, or pointed scissors to make a small hole in the blister [4]. It is best to be mindful of how the wound might drain, so a small hole at the base of the wound will help any excess fluid to drain with gravity [25] Once the hole is made, use sterile gauze soaked in saline or wound wash to gently compress the blister and let the fluid out [25]. If the roof of the blister flattens entirely to the skin, it should be possible to keep the skin intact, then follow “Care recommendation 21” If the roof of the blister has torn a little or the blister continues to fill with fluid, it is best to remove the skin flap and clean the wound with saline or sterile gauze [4,25]. Leave it open to let it dry out as much as possible, and absorb any excess fluid with sterile gauze Once the area has stopped exuding fluid and mostly dried out, mix a little zinc barrier paste (E.g. Sudocrem or Oxyplastine) with antibiotic ointment (E.g. Fucidin, Mupirocon, Bactroban, etc), and apply a thin layer to seal the wound This use of antibiotic ointment mixed with zinc barrier paste can be applied to intact or denuded blisters, and will help the wound to dry out and act as prophylaxis against any infection. If antibiotic ointment and zinc paste is not desirable for another clinical reason, it is also acceptable to sterilise the wound, allow it to dry, and then apply a thin layer of a basic
Written by Helen Lill: eicureproject@gmail.com21 Use cornstarch or baby powder to absorb moisture from wounds and stabilise the wound border [4,25]
barrier ointment (E.g. Vaseline, Aquaphor, 50:50) [4,25]. The objective is simply to disinfect, help the wound to dry, and seal it to reduce the risk of infection. EI skin proliferates at a higher rate than typical skin, and as such wounds can heal very quickly. A fresh wound can have healed skin within 6-12 hours if treated appropriately, and permitted time to dry out.
In order to keep EI skin clean and healthy, it is optimal to bathe daily, however this can increase the risk of wounds becoming water-logged and stop them from healing well. When wounds are present, it is still optimal to bathe, but to reduce the time, and to ensure that any denuded (raw) skin is protected with a layer of occlusive ointment before entering the water (See “Care recommendation 22”). To help reduce the moisture in a lanced blister, or to help dry and secure the edge of a denuded wound after bathing, cornstarch or baby powder is a useful tool [4,25]: 1) help soak up excess moisture with sterile gauze or a clean muslin cloth;
22 Once acute wounds from birth and associated handling have healed, commence daily bath routine with antimicrobial bath additive [1-6,8,11,21-24,26-29]
Written by Helen Lill: eicureproject@gmail.com2) put some cornstarch or baby powder into the palm of a clean hand;
3) use a clean finger to gently dab the cornstarch or baby powder onto the lanced blister and around the edge of a denuded wound; 4) wait about 5 minutes to see if any more exudate comes from the blister/wound, and repeat steps 1 to 3 if so; 5) once dried, the wound can be sealed with antibiotic ointment mixed with zinc barrier paste as described in “Care recommendation 20”.
EI skin is not able to shed in the same way that typical skin can because the stratum corneum does not detach as it should, and this is how the scale pattern is created in most forms of ichthyosis [1-3,5,6,8,11]. In addition the skin barrier function is impaired with EI, and this can mean that the skin is at increased risk of infection [1-3,5,6,8,11]. Bathing daily can help to keep microbial colonisation under control, and it can also help the skin to shed the stratum corneum to complete the skin cycle [1-6,8,11]. During the first few months of life, encouraging the stratum corneum to shed is less of a concern for a baby with EI, however establishing a good bathing routine and reducing the risk of skin infection are both important reasons to follow a daily bath routine [1-6,8,11]. It is important to add some form of antimicrobial agent to the bath (E.g. Octenisan, Dermol, or dilute bleach), and this will help to ensure any wounds are kept clean and minimise the risk of skin infection [21-24,26-29] It is worth knowing that denuded skin is extremely painful when bathing, so it may help to cover wounds with a little vaseline before going in the water. The vaseline will gradually melt away so that the wound is cleansed by the bath, but the pain will be minimised by this approach Baths in the first few weeks and months of life should be between 5 and 15 minutes, but as the baby gets older, the length of time spent in the bath each day will gradually need to increase to a minimum of 30 minutes to assist with the shedding of the stratum corneum [29] Typically scale starts to appear in earnest around 3-4 months, and at this stage, baking soda (bicarbonate of soda) or salt should also be added to the bathwater to soften the water, change the pH to be more alkaline (pH 7 9), both of which aid the shedding of the stratum corneum [29] See FIRST
23 Minimise use of soaps or shampoo [4, 21-24,26-29]
website for details on different types of bath: https://www.firstskinfoundation.org/bathing-exfoliation [29].
Soap and shampoo should generally be avoided for babies with EI because they are extremely drying to EI skin, irrespective of whether they are designed for use on sensitive baby skin Provided the baby is being bathed daily as described in “Care recommendation 22”, the skin will be adequately cleansed by water with the added antimicrobials at this age [21-24,26-29]. If there are particular areas that require more thorough cleaning, the use of emollient lotion on gauze works as an effective cleanser as described in “Care recommendation 13” [4].
24 Soak the scalp and gently massage to help scales/cradle cap to dissipate [1,5,10,11,17]
The scalp of any newborn can often be affected by cradle-cap, but for a baby with EI the scale seen on the scalp may be due to EI in addition to cradle-cap [10,17]. In either case, it is optimal to ensure the scalp is regularly soaked in the bath, and that this can be followed by gentle attempts to massage the scalp and exfoliate the scales using either a rough towel dry, or a special silicon exfoliator [1,5,11]. To ensure the scalp is soaked adequately, it may be possible to lie the baby flat in the bath so that the scalp is mostly under the water, or when the baby is supported in a bath seat/support, the scalp can be soaked by placing a wet washcloth onto the scalp for the duration of the bath. It is likely that it will take a few days to fully clear the scalp If the scale seems particularly stubborn, the scalp can be slathered in lotion and a cotton hat can be worn overnight to deeply soften the scale prior to repeating the above soaking/massaging care recommendation [1,5,11]. This may be done on consecutive nights to help to clear the scalp, and should be possible to maintain a good condition with once weekly applications thereafter [1,5,11].
Keratolytics such as urea, salicylic acid, alpha-hydroxy-acids, or propylene glycol are useful tools for managing hyperkeratosis [1-3,5-8,11,12], and any new parent with an affected child will think that they need to use these agents because so many affected individuals use them and talk freely about them within support-group social-media forums. However, it is extremely unlikely that a baby with EI under the age of 6 months has the type of scale that requires the use of keratolytics The onset of scale is typically around 3/4 months, and the need to start using keratolytics typically occurs around 12-18 months, but prior to 12 months keratolytics should be avoided [8,11]. The only exception is for babies with significant thickening of palms and soles due to PPK, and then this must be once per day and by prescription only [8,11] The use of keratolytics is safe when used appropriately, but it is important to be aware of absorption, and that it is possible to suffer mild to significant forms of toxicity from these agents if not used correctly [30-34] Whilst it is difficult for a parent or caregiver to watch the development of scales on an infant, the build-up of scales in these early months is unlikely to
Written by Helen Lill: eicureproject@gmail.com26 In the event of failure to thrive/weight-loss/failure to gain weight, supplement diet with high-calorie formula [6,8,35-37]
cause any significant infection risk provided a good bath and skin care routine is used, and as such the use of keratolytics should be reserved for when a child is older (12-18 months), and only with supervision from an experienced dermatologist [8,11].
Some babies with EI struggle to gain weight [6,8]. This is thought to be because of the higher calorific consumption resulting from wound-healing and additional skin growth [35,36] In the event that a newborn baby does not recoup their birth weight, fails to gain weight, or loses weight within the usual timeframe of newborn checks then this should be considered a red flag for dietary supplementation and referral to a neonatal dietician [8,37] It is also imperative that EI is not assumed to be the only potential cause for this failure to thrive, and that the usual clinical considerations are made (E.g. tongue-tie, GERD, food allergy, etc). In the event that a baby needs dietary supplementation with high-calorie formula, it is very likely that the child will require long term dietary supplementation, and appropriate professional dietetic support will be needed [8,37].
27 Supplement Vitamin D [8,35-48]
The potential for Vitamin D deficiency in EI is well studied and documented [8,35-43], and in fact those with EI may be at even greater risk than is seen with other types of ichthyosis [38] Vitamin D deficiency in children can lead to poor growth and bone deformity, with rickets found with relative frequency in children in some countries where access to routine supplementation is limited Vitamin D deficiency has also been associated with poor neuropsychological brain development, and has been linked to autism spectrum disorder in association with maternal vitamin D deficiency during pregnancy [44,45]. The mechanism behind vitamin D deficiency is not clear, it may be due to the presence of thick scales which could act as a barrier to UVB penetration of the skin [8]. Alternatively the impaired barrier function of EI skin might prevent proper vitamin D synthesis in the skin cells themselves [39]. Whatever the cause, it is important to take proper precaution to prevent vitamin D deficiency by giving adequate supplements to a baby with EI, and ensuring vitamin D levels are checked on a routine annual basis [46-48].
EI skin is not able to regulate temperature changes in the same way as skin which is unaffected by EI [8,11,49] It is typical for those affected with EI to report that they cannot sweat, or that they can sweat but it tends to be profuse and only from some parts of the body (E.g. face, hands, and feet) [50]. In a newborn, it is more common to see beads of sweat across the bridge of the nose In the event that sweat is observed on a newborn, this is a clear indicator that the baby is too hot, and measures must be taken to cool down [49]. It is also worth noting that EI skin becomes much more fragile when the temperature is higher, and as such general handling may cause even more injury than usual If a baby seems distressed and unable to calm down despite being clean, fed, and generally well-cared for, it is possible
Written by Helen Lill: eicureproject@gmail.com29 Minimise
they are simply too hot and are consequently feeling very sensitive. It is ideal to keep a thermometer in the area where the baby sleeps to help gain an understanding of what a comfortable temperature is for the baby. There is no ideal temperature as such, every baby adapts to their environment to some extent, however any extreme changes in temperature relative to the norm will require some adaptations. In hot weather, EI skin is more fragile so try to minimise handling, ensure a muslin cloth is always used when holding the baby to feed to ensure no skin to skin contact is made (skin to skin creates sweat and generates heat which may lead to blisters), use fans or air conditioning to ensure it is possible to stay cool, take care to ensure the car and/or car seat is cool when putting baby inside (park in the shade, run the AC for a few minutes before entering, or keep ice-packs at hand to cool the car seat down) [49]. At the other extreme, it is important to recognise that thermodysregulation and impaired barrier function means a baby with EI can get too cold more quickly also. This issue is typically first noted upon taking a baby with EI to the swimming pool, it is best to look for signs of cold such as blue-tinged lips, uncharacteristic crying, or shivering. In general, when first taking a baby to the pool, it is better to plan for a short time in the water, such as 10-15 minutes and then to gradually work on increasing the time.
Every baby with EI is different and their degree of skin fragility differs, so it is entirely possible that some babies will be able to tolerate the car seat, baby carrier, or bouncy seat much better than others. However, for some babies with EI, any time spent in these items will always lead to skin trauma, and the newborn period is when the skin is at its most fragile, therefore it’s important to be aware that these everyday items may in fact be the cause of blisters appearing on the legs, hips, thighs, and buttocks. It is therefore optimal to introduce these things slowly to a baby affected by EI Where possible, if it is desirable to use a baby carrier or bouncy seat, it is best to start with a 5-minute trial before checking to see if there is any obvious redness, and then gradually increase the time. For some babies with EI, their fragile skin simply cannot tolerate these everyday items, and as such it is best to lie them down onto a flat and soft surface where the pressure is more evenly spread. For many new parents, the car seat is a particular problem, and it is therefore recommended that journeys are kept short, and that long journeys are limited to those that are deemed essential
30 Look for signs of limited mobility and/or missing movement milestones, especially in babies with palmoplantar keratoderma (PPK) [51]
Written by Helen Lill: eicureproject@gmail.comMobility can sometimes be affected in babies with EI, and this is more frequently seen in those affected by palmoplantar keratoderma (PPK) [51]. When the skin is thickened on the hands and soles of the feet, it can sometimes feel painful to move, and as such there is often a delay in meeting typical mobility milestones in babies and infants with PPK specifically Signs of delayed mobility are not always associated with PPK however, so it is important to communicate any concerns with a medical professional, in order to ensure timely professional assistance for this issue Physical therapy can be utilised to help encourage proper
development, in addition to taking the usual measures to ensure the skin is kept as comfortable as possible. Despite the potential for trauma to the skin from handling, it is important to find ways to facilitate usual activities for newborns and infants such as tummy-time. Many new parents become afraid to allow their infants to move, but whilst this may reduce the risk of skin trauma in the short-term, it may cause bigger developmental problems in the long-term, so it is best to help parents find ways to adapt their environment and to cope with their understandable anxiety. This can be achieved through a combination of physical therapy, occupational therapy, and counselling where possible.
It is reported that 3-4 hours each day is typically required for the care of a baby with EI including bathing, applying creams and lotions, and wound care, in addition to all the demands associated with the logistics of procuring pharmaceuticals, managing care administration, and visiting medical professionals [1,17]. It is well-documented that those affected by a skin condition are affected psychologically by their condition [52], but it is only more recent that the psychological status of the parent or caregiver has been explored [53-55]. It is very clear that it is imperative that caregivers feel that they are supported from an early stage and beyond [53-55]. Whether this support comes in the form of counselling (online or in-person), financial aid, care support, or even with the assistance of a clinical care coordinator to advocate for the caregiver and baby, these are all examples of ways to help the parent or caregiver to process their experience of having a baby with EI, and to help support them to support their child to the best of their abilities [56,57]
References
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Written by Helen Lill: eicureproject@gmail.com