We’re starting a new project here, and if you have a form of epidermolytic ichthyosis, we’d like you to share with us and our readers how you take care of your hands and feet. We will collect, curate and then publish the responses on our blog here, with as much advice specific to each individual type of ichthyosis as we can.
Here’s how this project started. Last week, a friend with a toddler affected by epidermolytic ichthyosis (formerly known as epidermolytic hyperkeratosis, EHK, bCIE and other names) posted on a medical forum that she was having trouble dealing with her child’s feet. She told us she was unsatisfied with the responses from the panel of experts, because they only were able to tell her things like, “Well, generally, these creams…” and “ask your doctor.”
Rachel and I believe that as a community of affected people, we should aspire to have better information available. Now, I know we all have different mutations and differences in what our skin looks like and how it responds to various treatments — so as always, we have to be careful to pick out the relevant advice and disregard the rest, because what may be good advice for one person might be catastropically damaging to another. And we use different creams depending on our location and what our medical insurance will cover. I think we all understand that.
But just because there’s a wide range of different types of ichthyosis, and a wide range of treatments, it doesn’t mean that we need to keep the discussion at such a general level that it’s difficult for someone looking at the responses to figure out what might be applicable to them. And we also think the reality is, the affected families are the ones with experience, so this level of detail is only going to be available from affected families.
Each of us had to figure out, individually, what worked for us. Why can’t we share that information? So please, step up here to help out the new moms who might be just starting to invent their own wheels.
Below the jump, I have pictures of different types of keratinizing ichthyosis. Please tell us which one looks like you, and how you cope with it. And remember, we are looking for specifics tied to your specific type of ichthyosis.
(All pictures used here with permission.)
1. Epidermolytic ichthyosis / KRT10 (clear palms and soles, blisters elsewhere).
Let’s start with the type of epidermolytic ichthyosis that usually has problems with blisters all over the body, but with the palms and soles of the hands and feet staying relatively clear, with maybe a little heel or palm thickening if it gets neglected.
If your hands and feet looks like this, how do you manage it? How often does it blister? Does it come off in sheets or does it just keep growing thicker? Do creams sting? Do acid lotions help? What about petroleum-based creams like Aquaphor, Vaseline and 50/50 paraffin?
2. Epidermolytic ichthyosis / KRT1
Next, we have pictures of a baby with the KRT1 form of epidermolytic ichthyosis. Her mom says putting any socks or shoes on causes her feet to blister, and her hands blister any time they get hot. Mom says that lotions burn and that she’s constantly dealing with a cycle of rawness and blisters, popped blisters, then rock hard dry yellow skin. The baby seems to go through seasonal skin shedding.
If you have the KRT1 form of epidermolytic ichthyosis, is this what you’ve experienced? How do you deal with the blisters and rawness?
3. Pachyonychia congenita
Pachyonychia congenita is also a keratin disorder, but it’s involves a different mutation than the ones in the ichthyosis “family”. It affects only the hands and feet, and shares symptoms with palmoplantar keratoderma (PPK, a type of epidermolytic ichthyosis caused by mutations on the KRT2e gene). Rachel and I are less familiar with it, but we’ve seen common advice that seems applicable, so we wanted to include it here.
These photos of someone with pachyonychia congenita are from our guest post a few weeks ago. You can see that it is so thick that she can barely bend her fingers. After she filed it with the Dremel, she got some mobility back. If you have this particular form, are your hands this thick? What sorts of creams or files or acids help you?
4. Ichthyosis en confetti — type 1
The “Type 1″ form is what most people with “ichthyosis en confetti” have. The owner of the hands pictured here says that a little Aquaphor and gloves is all she really needs to manage them, without filing, but that her nails are thick and hard to trim.
5. Ichthyosis en confetti — type 2.
This is what Rachel and our boys kids have. So far as we know, we’re the only ones with this type. (Anyone else out there?)
The hand in this photo is a picture of Cookie’s hand, from before he was really old enough to do much filing and maintenance. His fingers still get like that, especially in winter, but the palms generally aren’t that thick when we keep them filed. Even with filing, they tend to have open cracks at the base of their thumb and along the pinky side of the palm and across the wrists. The feet look about the same, with really thick buildup and cracks around the heels and along the sides near the toes. The middle of the sole stays pretty clear.
We’ve written quite a bit about how we cope with it. Our favorite tools are heavy black files, a cuticle nipper, the Dremel, Aquaphor and Glad Cling Wrap. We try to keep their shoes and socks on all the time to keep their feet soft enough to not crack. They pretty much exclusively wear velcro sneakers. All those cracks make tie shoes difficult, and slip-on, leather or high top shoes are hard to get over cracked heels. The boys tend to have gait issues, so open-backed sandals or flipflops with the toe loop don’t work because they tend to fall out of them, and the toe loop creates blisters between the toes.
So….if you have one of the types of ichthyosis shown here, please share with us whether your hands and feet look like what’s pictured here, and whether you have any additional skin-care tips for your hands and feet that have worked well for you. Please be as specific as you can.
If you leave a comment on this post, we may include your comment on our followup compilation. We’ll also collect responses via Facebook, via email, and any other way you can get information to us. We’re happy to keep submissions anonymous if you’d like us to.