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"Confetti Skin, Beauty Within" is our blog about ichthyosis and its effect on our lives. Rachel and our three boys are affected with the form of ichthyosis called "icthyosis en confetti, type 2".

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Here's our summary of our best and most important posts of 2012.

Epidermolytic Ichthyosis: Hands and Feet?

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.

KRT 10 hand-001

KRT 10 foot-001

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?

KRT1 sole-001


KRT1 EI hand-001
krt1 blister hand-001
KRT1 fingers-001

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.

pachyonychia congenita 2 wm

pachyonychia congenita wm

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.

confetti type 1 hand-001

confetti type 1 palm-001

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?)

confetti type 2-001

 

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.

4 comments to Epidermolytic Ichthyosis: Hands and Feet?

  • Sarah

    I have type 1 – EI/EHK/non-bullous CIE (whatever)
    Feet blisters are almost constant with the area where toes meet foot the most affected, this is where shoes crease when walking. Eventually the blisters go down and peel off. The peeling is usually in large chunks, not sheets. Cracking around the ankles is especially painful. No scrappy sandals or cute high heels for me. Foot blisters are my number 1 hurdle, followed closely by heat issues.
    Hands don’t seem to have as much due to less chafing. As a string player getting and maintaining callouses is quite difficult but that doesn’t stop me from playing my cello or viola. I also play large, bass handbells that can cause blisters where I grip the bell handle. Once blisters form and peel a callous can be formed but it isn’t always comfortable.
    Acid based creams simply sting too much so I use less painful lotions, almost anything will do. Sometimes I use the thick Eucerin or aquaphor on my feet and hands but that is quite rare. I do take Soriatane so my skin isn’t as thick and a little more fragile than it would be naturally.

  • Carolyn

    I have Type 1. My palms & the soles of my feet are clear. As I type this it’s ‘spa night’ at my house. :) Here’s what I do:
    3 times a week put on any old lotion (store brand works just fine) and then put on a sauna suit for a couple of hours. You can buy these in the exercise section of stores like Walmart & Target for around $10.

    After the suit, I shower and CAREFULLY scrub my skin, then put on more lotion.

    Daily regimine is quick shower in the morning and Cetaphil cream. I use Cetaphil because it’s fragrance free and lasts me all day.

    Evenings, when it’s not spa night, are a shower where I use the rough side of a Scotch-brite kitchen scrubber instead of a washcloth.

    Creams generally don’t sting unless I get them on raw spots or blisters. Overheating & blistering are problems but after this much time it’s just how things are. :)I use no prescription creams or drugs at all. I outgrew serious infections when I was a teenager. Everything that pops up I can manage with Neosporin.

    Hope this helps!

    Thanks for all the work you do!

    Carolyn

  • Toni W.

    I have type 3. It’s a bit different in the sense that it doesn’t ever change. The pictures above are not photos of a “break out” or “flare up.” That’s just what it looks like. It never spreads, shrinks, or changes at all.

    Walking is my number one obstacle. There are many times when I can barely stand but I can usually walk if I have a wall to lean on to. When it gets wet, it can become “spongy” making that time the absolute worst to walk. I bathe at the very end of each night because once I get out of the water I will not be able to walk anymore for quite a while.

    I use a lava rock (kinda like a pumice stone but more abrasive) and have had great results with a dremel. I’m prescribed lactic acid (12%) but the results are pretty minimal. I also use Bactroban and sometimes Neosporin to put over really deep cracks because they can (and have) become infected. The worst spots for infection are obviously the cracks which can be very deep at times. This is because it’s so thick that it really hinders my ability to move my hands. They do not open or close fully. If I don’t take care of them they will become so thick that I can’t move at all which makes it WAY more painful to crack so I can move.

    Another big obstacle is movement, of course. Because they do not open or close all the way, I have very little dexterity and I have trouble with tasks that require hand strength, such as opening jars, writing with a regular pen, or anything else that would require me to grip something with my hands. I use a piece of rubber to open jars. It’s a sheet and it grips for me since my hands can’t. I also have a tool that closes around lids and has a handle so I can use that to open stuff. It also has a clip on the other end for things like pudding cups and pop cans. I highly recommend it to anyone who has kids going to school. It helped me a lot to feel independent and not to always have to ask someone else to open things for me. The tool would fit nicely in a lunch box.

    It comes off in chunks varying in size but they are pretty large and so I do sweep quite a bit. I don’t have any sweating/heat problems but it does appear that I’m able to sweat from “under” that skin, causing pockets and blisters to develop under it. I’ll try to get some pics next time that happens.

    When it’s really bad or thick the best thing I can do is coat my hands and feet REALLY THICK in ointment, lotion, or udder cream then put gloves and socks on and sleep that way. It helps a lot to have it on for enough time to soak in because when I just rub lotion on it doesn’t absorb right like the rest of my skin does. Where the “dead” skin meets the normal skin can hurt a lot too. In the pictures you’ll notice it’s red. Sometimes the “dead” skin will peel and go straight into the live skin so it rips and hurts. I have to be careful for that. Also when I was a little kid my hands and feet (esp. feet) didn’t hurt quite as bad. I’m sure it has something to do with the fact that now I’m bigger and heavier than I was as a kid.

    I hate shoes. Seriously, I will go really far out of my way not to wear shoes. If it wasn’t a legal thing (and gross) I probably wouldn’t even wear them in public. They make my feet swell and sweat causing the skin to get soft. They are rarely ever comfortable. Really, I hate shoes. They’re awful. The ones that are closed in are worst and high-heels aren’t even a possibility but flip-flops work for me. I also have a bunch of house shoes/slippers that I buy in different colors and styles so I can still match my clothes. Flip flops are what I wear most often though. They are very comfortable and I think it’s because they’re not closed in and they do not make my feet sweat (causing them to get wet.)

    Also, I occasionally get big blisters other places on my body and they’re super painful but those come and go. I haven’t found a way to deal with them yet so if anyone has ideas let me know.

    I’ll add more later if I can think of anything.

  • Pamela Blubaugh

    I have #4 en – confetti type 1. My palms and soles of feet are clear. I attended the conference in Indy and found in my goodie bag a product that is amazing. DerMend alpha+beta hydroxy therapy. This product stings a little at first but to be able to apply to my entire body right after a bath and be able to put clothes on is a blessing. I have always used vaseline or aquaphor. These products make me feel better but in order to dress I have to wipe the biggest majority of it off ( defeating the purpose). With the Dermend alpha+beta hydroxy therapy cream a little goes a long way and I do not have to wipe it off. Please try this product and let me know if it worked for you. I will warn that if your palms are clear, I recommend you wear gloves to apply to the rest of your body. This product also has a sun warning ( be very careful). This product not only changes the appearence of the skin scales, it also leaves you feeling completey moisturized. Little to no oder. For parents who are not affected trying to help their children I suggest that you treat them as if they were a burn paitent. When bathing make sure the wash rag has plenty of product or soap and be as gentle as possible. My skin hurts to touch so anything that is cold hurts. When applying product, rub it in your hands first to warm before applying to your child. Again, applying very gently. If going out to work in the yard I soak a white long sleeve shirt in cool water, ring it out and put it on. Not only does it keep me cool, protects from sun it also keep the skin hydrated making it easier to remove in bath or shower. For the comment above. I too get big clusters of blister like sores that cvome and go. They are very painful and do not seem to be filled with anything, tend to appear on eyebows, nose, ears and on back right above buttocks. I use preperation H ( Yes the hemorrhoid cream)it takes the pain and swelling from these blister like sores away. Hope this helps.

    GOD BLESS
    Pamela Blubaugh

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