Redefining the role of barrier ointments in epidermolytic ichthyosis: a multipatient perspective on the benefits of baking soda baths and ceramide lotions
Introduction
The benefits of ceramide lotions for restoring an impaired skin barrier have long been recognised in atopic dermatitis [1], and have more recently been acknowledged to be beneficial for the ichthyoses [2,3]. However, despite academic recognition that barrier ointments may cause an increase in blistering and the incidence of skin infections in epidermolytic ichthyosis (EI) [2,3,4,5], barrier ointments such as vaseline, aquaphor, and 50:50 continue to be prescribed as the mainstay moisturiser for these patients. Whilst these ointments certainly maintain an important role in the care of wounds in EI, it is important to highlight to the dermatology community that occlusive ointments should not be routinely prescribed or recommended for use as a general moisturiser for EI [2,3,4,5].
Basic principles of skincare for EI
EI is characterised by hyperkeratosis predominantly on the skin flexures and folds, in addition to blistering and skin fragility associated with heat, humidity, or light trauma [3,4,5]. The barrier function of the skin is impaired, and affected individuals frequently suffer from local or systemic skin infections [3,4,5]. The stratum corneum is unable to shed normally, and consequently the characteristic horny layer forms, and must be removed with assistance from regular bathing and manual exfoliation techniques [3,4,5].
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 [2]. 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 [2].
When barrier ointments are used as moisturisers for EI, the ointment reduces the efficacy of bathing, blocking the stratum corneum from shedding, and this leads to further thickening and darkening of the skin. In addition, the high occlusivity of ointments increases both heat and moisture in the stratum corneum, which increases its instability leading to blisters and wounds, and creates the perfect environment for bacteria and fungal infections to spread [3,4,5].
The following patient case studies demonstrate how EI skin can improve when using a care routine including daily bicarbonate of soda baths followed by application of ceramide lotion (Aveeno Dermexa/EczemaTherapy) in comparison to either a standard non-ceramide emollient or an occlusive barrier ointment.
Patient 1: 3/4 month-old baby girl, cool climate, 50:50 ointment daily since birth
The mother sought advice regarding patches of thickened brown hyperkeratosis (Fig 1, 1a), in addition to her daughter waking in the night and seeming to suffer with itch. In discussion, it was ascertained that the night-time room temperature and breathable cotton clothing were appropriate, however potentially heat and subsequent itch may have been caused by the use of occlusive 50:50 ointment prescribed for daily use. In addition, a regular bathing routine was yet to be established, with the mother feeling afraid that bathing would dry the skin further. On account of the brown scale, and night-time itch, the mother was advised to try a short baking-soda bath before bedtime, followed by application ofAveeno Dermexa/Eczema Therapy. If the baby was unable to lay in the bath, the mother was advised to soak a washcloth in the bath and lay it over the chest to help the scale to soak and soften. The mother was advised to gently massage the lotion into the scaly areas to help encourage the scale to shed.After 1 day, the skin started to improve (Fig 1, 1b), and after 1 week, the dark scale had cleared (Fig 1, 1c), and the mother reported the baby was sleeping better at night with no evidence of itch.
Patient 2: 6 year old boy, hot dry climate, vaseline daily since birth
The mother sought advice for her son’s overall skin condition. He was regularly blistering, suffered frequent infections, and had very darkened, thickened and painful areas of scale (Fig 1, 2a). In discussion, it was ascertained that a bathing routine was in place, but it was culturally typical to have a hot bath which was likely increasing the tendency to blister. Since birth, the dermatologist had recommended Vaseline be applied liberally as required. The family lives in a hot and dry climate, and techniques to avoid heat with the aim of reducing the frequency of blistering were discussed. However, it was high-lighted to the mother that a reduction in thick skin would help to reduce heat intolerance by allowing the skin to breathe/sweat more easily, and subsequently reduce the frequency of blistering and infections. We explored what resources were available, such as using an emollient instead of vaseline, and that bathing daily with either salt or bicarbonate of soda in the bath would help to soften the skin before gently exfoliating with a washcloth or sponge. The mother confirmed it was possible to acquireAveeno Dermexa/EczemaTherapy, and was willing to try this following completion of a 3-day patch test.After 1 day of proper use, a lot of the thick scale on his back started to slough away (Fig 1, 2b), and after 1 week the dark and scaly skin had almost disappeared (Fig 1, 2c), blistering had improved, and his skin was more smooth and comfortable.The mother also reported how much happier her son was with his skin. The family were thinking of leaving their home, because they were being treated like social outcasts. Once the skincare routine was changed, the boy felt more comfortable and confident, and their lives improved dramatically.
Patient 3: 5 month old baby girl, Dexeryl emollient daily, changed to ceramide lotion Aveeno Dermexa/Eczema Therapy bi-daily for 3 days
At presentation, the yellow scale had started to increase in the joints and flexures around 4 months of age, and continued to progress (Fig 1, 3a-3c). The skincare regime included a daily bath followed by application of emollient lotion, Dexeryl. Whilst this lotion is easy to apply, and allows the skin to breathe, it does not contain ceramides, so the mother was advised to try to add a ceramide lotion into the routine. Following a 3-day patch-test,Aveeno Dermexa/EczemaTherapy was applied bi-daily for three consecutive days. No attempts to exfoliate the scale beyond gentle massage were advised because infant skin is too fragile, so the reduction and smoothing in yellow scale shown in the images (Fig 1, 3d-3f) was achieved only with the application of ceramide lotion.
Summary
This multi-patient perspective demonstrates the importance of recognising the variety of care needs for the different types of ichthyosis. Multiple sources reference the potential harm that can be caused by the use of occlusive ointments for EI [2,3,4,5], and many sources demonstrate the potential benefits of ceramide lotions for improving and soothing an impaired and inflamed skin barrier [1,2,3]. Despite this, many of the EI community continue to be told that they must use ointments to moisturise the skin, only to be told that dark scale and frequent infections are all part of having EI. I would like to appeal to healthcare practitioners to encourage patients to try new lotions, and to consider that dark scale, frequent blistering and infections may be improved by a change in skin care regime. The understanding of basic principles of skin care, including regular bathing and the use of modern ceramide lotions as demonstrated here, can be life-changing.
References
1. Chamlin, S.L., Kao, J., Frieden, I.J., et al (2002) Ceramide-dominant barrier repair lipids alleviate childhood atopic dermatitis: changes in barrier function provide a sensitive indicator of disease activity. Journal ofAmericanAcademic Dermatology; 47 (2): 198-208.
2. Foundation for Ichthyosis and Related Skin Types - FIRST(2022) Types of Ichthyosis: Epidermolytic Ichthyosis, a Patient’s perspective, and Bathing and Exfoliation. Available at: https://www.firstskinfoundation.org/types-of-ichthyosis/epidermolytic-ichthyosis and https://www.firstskinfoundation.org/bathing-exfoliation.
3. Suessmuth, K.,Traupe, H., Metze, D. et al (2020) Ichthyoses in everyday practice: management of a rare group of diseases. Journal of the German Society of Dermatology: 225-243.
4. Rout, D., Nair,A., Gupta,A., et al (2019) Epidermolytic hyperkeratosis: clinical update. Clinical, Cosmetic and Investigational Dermatology; 12: 333-344
5. Mazereeuw-Hautier, J., Hernandez-Martin,A., O’Toole, E. (2018) Management of congenital ichthyosis: European guidelines of care - Part One. British Journal of Dermatology
