This online discussion session with Dr Will Davies (Senior Lecturer, Division of Psychological Medicine and Clinical Neurosciences, Cardiff University), Georgina Wren (research student, Cardiff University), Mandy Aldwin-Easton (Director and founder member ISG), Sarah Griffiths-Little (ISG Director), Ray Rowley (ISG Vice-chair) Sue Corbett (ISG Director) and a number of ISG members attending by invitation.

Mandy (who has ichthyosis) had arranged this session with Dr Will Davies, offering ISG members an opportunity to discuss their experiences and concerns in relation to ageing with a variety of forms of ichthyosis with expert advice on hand.  Sarah and Ray of the ISG (both living with ichthyosis) were attending as members and Sue (ISG secretary and not affected by ichthyosis) was invited to take notes.

Researchers

Will Davies introduced himself and explained that he has had an interest in ichthyosis for some time – developmental and psychological issues – in particular Ichthyosis vulgaris and X-linked ichthyosis.  Will and his team have received funding for an undergraduate student to work through the summer looking at ageing related issues in various forms of ichthyosis and how these issues may impact upon people’s lives.  He is keen to identify key research questions that those affected would like the research directed towards.

Georgina Wren explained that she is in her 2^nd year of a 4 year PhD course, looking at neurological and mental disorders.  She has also looked into mental health and psychological issues surrounding endometriosis.  Georgina will be hosting the undergraduate student though the summer and is keen to find out the questions that will help form the work for the summer.

Please look out for the research survey that will be distributed during Summer 2022.

The Discussion

As knowledge about and treatment for the various forms of ichthyosis improves, along with the widening variety of creams and emollients, those living with the condition are able to look forward to a longer life than their predecessors with the condition, so discussions around ageing with ichthyosis have not previously happened.

So, the question was put to those participating – ‘ How has your skin condition changed with ageing and what are your concerns for the future?’

Here is the information that was shared:

For anonymity, the ISG members attending are not being named, but are here identified as ‘Member A’ etc.

Member A – 46 year old female with Congenital Ichthyosiform Erythroderma (CIE).  Reports that her skin still heals very quickly, although her skin condition is worse in general with age.  Has noticed joints aching over the past year.  Feels vitamin D is very important in a diet, but this has only been acknowledged by the medical profession in very recent years. So, would her skin have improved if additional vitamin D had been suggested earlier in her life - ?  Member A was put on antihistamine from the age of 18 months until 15 years old named Vallergan.  This led to bad withdrawal symptoms and depression that lasted about 10 years.  More recently she has tried antihistamines again and found that they help – but Vallergan proved to be the wrong type of antihistamine.  Following that she started smoking to ease the stress she felt – and still does.  As a child the family had a water softener, from age of 18 (presumably the family moved house) they had hard water and this appeared to make her skin feel worse.  Has taken Acitretin since the age of 7 years and wonders whether Acitretin’s effect wears off with age as it doesn’t appear to be as effective now as it was when younger.  Has quite severe hair loss and believes this to be a side effect of Acitretin.  Has never had more than 20mg (she had hair loss and constant nose bleeds).  Recently has reduced her daily dose to 10 mg as she worries about the long term effect on her general health and especially her liver.

Member B – female in her mid-forties with Netherton syndrome.  Her skin healed very quickly when she was young, but not so quickly now.  Takes Naproxen for pain relief when required. Has noticed arthritis type symptoms in some joints since turning 40 but hasn’t had a diagnosis.

Member C – female in her early fifties with Congenital Ichthyosiform Erythroderma (CIE).  Has been on medication (Acitretin) for her skin since the age of 16.  Was prescribed a high dose (50 mg) from age 16 to early 20s.  Has noticed that when she gets a break in her skin, or a shoe rub, it now takes longer to heal – sometimes even the smallest break can take 2 weeks to heal, despite maintaining good hygiene to the area.  Currently managing on 30mg of Acitretin – any less causes her feet and hands to flare up.  Has noticed brown patches on the skin of her lower legs (only) – very similar to ageing spots on people in their seventies.  Has severe hair loss (wears a hair piece) and reports she has no body hair.  Has had open heart surgery twice – her surgeon told her that her heart issues were problems around her heart, probably linked to her skin condition.  (Was told that, as her skin is affected, so too is her heart – possibly all her body organs: however, nobody else has been given this advice.)  More recently she has been diagnosed with Ankylosing Spondylitis (inflammation of the spine and other areas of the body) which causes stiffness in her lower back and hips.  Stopped taking Acitretin 2 years ahead of starting a family.  Her skin suffered badly.  She had a second child soon after the first and then resumed taking Acitretin.  She carried both babies to term and had normal deliveries.  Neither child has the condition, but they are both carriers of the gene.  During this period not taking Acitretin she felt very vulnerable and isolated because of her appearance and she suffered depression.  She found that people were judging her on her appearance alone.   As a child she suffered bullying at school and became quite withdrawn.  However, Acitretin changed her life and she went through college then university, gaining confidence.  She credits Acitretin with her success at work.  She never received support at home from her family and siblings, so has turned to her friends in the ichthyosis community who completely understand her situation, worries and fears.  In very recent years she feels she has been going through the menopause as she has suffered night sweats (most unusual), but blood tests reveal that she isn’t.

Member D – male in his early fifties with Epidermolytic ichthyosis (EI).  Took Acitretin in early life, but stopped taking in his teens.  No medication now.  During lockdown he found he had more time for a better skin care regime as he needs to exfoliate regularly, but carefully.  With this condition the skin thickens, so in order to maintain flexibility around his joints he needs to exfoliate regularly.  His concerns are that he needs to maintain physical flexibility in order to maintain his skin.  He feels the biggest concern with this condition is overheating due to much reduced ability to sweat.

Member E – female, aged 35 with Netherton syndrome.  Currently taking 30mg daily of alitretinoin.  Currently has 2 small cuts on her feet that are struggling to heal, possibly due to her medication, although she has only been taking this medication for 6 months.  This problem with her feet has been on-going for 14 months and is being monitored by weekly visits.  Her skin’s healing ability changed around the age of 25 years.  She also suffers severe hair loss.  She is not diabetic and her blood flow is fine.  She has severe allergies (this is quite common with Netherton Syndrome) and gastric problems.

Member F – female, 42 years with Lamellar ichthyosis (LI).  Has been taking Acitretin since the age of 2.  She alternates between 20mg & 30 mg daily.  She has found that her skin is improving with age, but feels that this may be due to a better self-care regime that she has adopted.  She has noticed aches and pains in her knees but was reassured by her GP that this is probably due to lockdown weight gain and not her skin condition and so is working at losing weight.

Member G – female, 40 years old with Ichthyosis en Confetti.  This member has had a number of surgical procedures to remove melanomas.  Her skin’s healing process is still quick, but she feels her skin is drying quickly with age.  She first noticed her skin drying at around age 25.  She finds that her baths no longer give her the comfort they once did.  She is now noticing a number of seborrheic warts appearing.  Her skin care regime is very time consuming and she has found during her many periods in hospital that the nurses don’t have the time to help her with this and so her husband has needed to visit to do this for her.

While this hasn’t yet been a problem, she worries that a time will come when she will be required to stay in long term care and will get no support with her essential skin care.  She already feels that her body is ageing rather quickly and her skin is deteriorating at speed.  Her skin is getting redder with age.  At 40, she feels ‘old’ – she has recently been diagnosed with osteoarthritis in her knee – severe wear and tear.  She reports that she has had a very short attention span for a few years.  During her recent pregnancy she found her skin was ‘good’ and she felt better.  Recently she has found that her scales are smaller and more adherent.  Her physiotherapist tells her that her muscles are ‘fibrous’.  Is there a connection?

Member H – female, 52 – with Netherton syndrome.  This lady lives in the Netherlands and is currently taking part in a research group there.  As part of the research she has received sunbathing therapy, which she found helped her skin (as she lives in the Netherlands, there is naturally little opportunity to sunbathe).  Also, pain management therapy where she was taught that pain increases with stress levels.  So, to reduce pain, look to reduce stress.  She has been diagnosed with osteoarthritis and she has an underactive thyroid.  She reported that, since the age of 35 she has noticed small brown areas appearing across her body – they started on her hands and face but have since spread.

General notes:

In stressful situations and at stressful times, the skin is worse for some people.

During a woman’s menstrual cycle, the skin may be worse.

No woman participating has gone, or is going through, the menopause.  However, they have concerns about such things as night sweats – as people living with ichthyosis don’t sweat: what will happen? How will my skin cope?

Pain management – 2 members had been offered advice on pain therapy from the NHS.  Both ladies reported dreadful experiences due, they both felt, to the lack of knowledge of their severe skin condition.  One member found it extremely hard to talk about her experience as she is still traumatized 10 years later.  The second member was left feeling exposed and vulnerable from her experience.

Other remedies members would like investigated:  the legal use of CBD oil.  This is used in the Netherlands and has proved successful.  Could it be added to skin cream?  There has never been much consideration given to pain management with ichthyosis in the NHS – presumably simply because it is such a rare condition.

Temperature fluctuations – cold feet!  Even in warm conditions.

Concern expressed by some about the physical aspect of caring for the skin when less mobile, or unable to in later life.

Biologics – this form of treatment is being used to treat some skin conditions, but there is currently limited funding in the NHS for trialling it with ichthyosis.

The meeting ended around 9.00pm.  Everyone was thanked for their participation, openness and honesty about their situation and concerns.