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#28820 - 02/23/13 07:28 AM Help!!
imranym Offline
Member

Registered: 02/23/13
Posts: 5
Dear All,

We've just had our second child (5 weeks old) who was born as a collodion baby boy. I have an elder daughter who is now 3.5 years with no signs of ichthyosis. My wife and I are both unaffected.

With 5 weeks gone, the membrane has only partially peel off. It has peeled off from the neck, torso and hips. Parts of his face, all arms and from knees to toes are still covered in the membrane. his second layer of skin has also started to surface on his torso and face which has now turned a bit dark (although no signs of scale formation yet) and started flaking.

Can any parent/affected individual who has gone through this process help describe what is going to happen next?

Is the first layer of skin the worst that grows immediately after the membrane sheds?

Typically what age does scaling start (if it does at all)?

Are there any examples of children who have completely recovered from a collodion membrane?

Any help is most appreciated. We're residing in Saudi where local doctors have very little exposure related to ichthyosis and thus have been not as helpful as one would hope.

Regards,

Imran - concerned parent facing this for the first time.

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#28821 - 02/24/13 09:16 AM Re: Help!! [Re: imranym]
Hearsay Offline


Member

Registered: 01/16/04
Posts: 1449
Loc: Richmond, VA, USA
Hi and welcome.

The collodion membrane is usually a signal of a recessive disorder. Basically, you and your partner are both carriers of the gene, and you have a 1 in 4 chance of each child being affected. Your older daughter is either a carrier like you both, or is not a carrier. The recessive disorder manifests on one of 2 ways - CIE, short for congenital ichthyosiform erythroderma, which is usually a lifetime of reddened, flaky skin and sometimes hair loss, or lamellar ichthyosis, which tends to have bigger plate scales that don't really come off easily.

There are several genes that cause these disorders. Most of them are known and can be tested by a lab in Maryland called GeneDX. There should be other places in your part of the world as well that do the testing. The most common gene is a TGM1 mutation, but there are several others as well.

For the most part, whatever level of scaling you will deal with will stabilize around 6-12 months. It can get worse if it is neglected, and better with diligent care, but it will never cure. There are a few cases of self-healing collodion baby, where the membrane sheds and the baby has normal skin afterwards, but it pretty rare.

Things you want to watch for: eye damage. The eyelids get pulled down by the dry skin so they can't close completely. Dry corneas become ragged and make vision blurry. It's very slow to heal and can be permanent. Use eye drops during the day or ophthalmic petroleum at night.

Ears - they tend to build up with wax and skin and get completely blocked. You will have to visit a doctor to help you learn to care for this. Some people use olive oil drops or medicated oil drops to loosen scale. We use a 50% hydrogen peroxide/water mix and flush the ears out every other week or so.

Overheating - the thick skin is like an extra layer of clothes. In hot weather, overheating is a real concern. This is worse in really high humidity and in really dry areas.

Dehydration - the skin barrier is broken, so you will find that the child will be thirsty and drink more than normal. If they overheat or get a fever, they are more likely to face heat exhaustion or heat stroke and may need IV fluids.

Lotions - generally creams work better. Vaseline is ideal, but it makes a mess, so it's a balance between comfort and sanity. Acid lotions help remove the scale, but most aren't thick enough to help with the water barrier problem. So you'll probably find that an acid lotion AND a thicker cream work best.

Generally, finding a knowledgeable doctor means finding a teaching hospital attached to a big university. Most local doctors have no idea what they are doing. But even then, most aren't much help beyond the initial diagnosis. I've always found the best support comes from other parents.

There's an active group on Facebook that might be of interest to you. Several internationals are members and you might find someone in your region that could help navigate the local doctors.

_________________________
Jennifer
Ichthyosis-en-Confetti Type 2
Husband, Nathan - 10, Elliot - 7, Oliver - 4, all affected.
I also have an unaffected daughter, age 8.


email: jennifer at confettiskin dot com
facebook - find me on "ichthyosis mommy spot" or "friends of ichthyosis"

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#28822 - 02/25/13 12:52 AM Re: Help!! [Re: imranym]
imranym Offline
Member

Registered: 02/23/13
Posts: 5
Hi Jennifer,

Thank you for your reply. The information I have received from doctors has been very vague and conflicting between the different dermatologists. I have also come to know through various sites/forums that the best source of knowledge is from different parents who have gone through this before.

In essence I’ve taken feedback on Abdullah’s skin from as many as six different dermatologists here available locally and they’ve had varying and conflicting views which has just added to our concern and confusion.

Abdullah's current state is that the new skin surfacing turns slightly dark and then starts flaking. This has happened once on his lower torso (currently happening for the second time) and is happening for the first time on his face. His back has become a bit rough but nothing seems to have shed off - shortly after birth his back skin was a smooth as a normal newborn. The membrane has started to crack from his legs and arms at this stage.

We have been applying baby vaseline every four hours and have kept his eyes well lubricated with artificial teams. He has now stared to close his eyes properly and the ophthalmologist was satisfied with his progress.

Based on your feedback, i would be taking Abdullah to see a doctor specifically for his ears.

Doctor 1
• Not a Collodion baby
• Affected moderate to severe due to the presence of ectropion
• Probably Lamellar
• Revisit in 6 weeks
• Administer Acetritin in controlled dosages once baby is +2 months in age.
• Premature to state his severity levels at this stage

Doctor 2
• Collodion baby
• Severely affected
• X-Linked (although family history suggests my mother's cousins having bullous ichthyosis)
• Will affect all males in future babies
• Not required to revisit – manage through ointments
• No possible recovery. Will remain lifelong with scales all over the body

Doctor 3
• Collodion baby
• Mild to moderately affected
• Scales will not develop
• Bi-weekly visits
• High chances of recovering completely within 6 months

Doctor 4
• Collodion baby
• Not severely affected
• Not required to revisit
• Will recover however it may take time (did not mention how long)

Doctor 5 (only through pictures)
• Collodion baby
• Premature to state if it would develop into ichthyosis.
• Advised to wait and see

Doctor 6 (only through pictures)
• Collodion baby
• Affected very mildly
• Chances of recovery are high


Warm Regards,
Imran

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#28823 - 02/25/13 09:16 PM Re: Help!! [Re: imranym]
Hearsay Offline


Member

Registered: 01/16/04
Posts: 1449
Loc: Richmond, VA, USA
Ok, of those, Doctor #1 sounds like he has a clue. (although it sounds like he's confusing collodion with harlequin scaling) However, I would not recommend acitretin in a 2 month old. Previous steroids have had major side effect issues. This particular one is fairly well understood in the short term, but the long term effects remain unknown. If acitretin meant life or death, as in a harlequin newborn, then it would be worth the risk. But for a lamellar child, it's mostly a aesthetic choice.

Definitely come find us on Facebook. This site is largely quiet nowadays, but there are loads of lamellar families around that can give you better advice than I can. I will try to direct someone over here.
_________________________
Jennifer
Ichthyosis-en-Confetti Type 2
Husband, Nathan - 10, Elliot - 7, Oliver - 4, all affected.
I also have an unaffected daughter, age 8.


email: jennifer at confettiskin dot com
facebook - find me on "ichthyosis mommy spot" or "friends of ichthyosis"

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#28824 - 02/25/13 09:59 PM Re: Help!! [Re: imranym]
Traci Offline
New member

Registered: 02/25/13
Posts: 1
I have Lamellar Ichthyosis, I am 41 years old. I was born with it, I was not born in a collodion membrane. Mom said my skin was red and tight, my eyelids were rolled back when I was born (but they did go down). I was taken to the hospital for 16 days. My skin was flaky and got darker as I got older. As a baby mom would put aquaphor on me and put me in plastic wrap to keep my skin moist.

As I went through school my skin was really dry and would crack. I lost most of my hair and I wear wigs now. As I got older my skin lighten a little. I use lac-hydrin which burns with cracks, but does help. A lot of my friends affected by Ichthyosis uses Eucerin creme.

I have a sister who is not affected by Ichthyosis and she has two children who are not affected either. I did not have any children. I am married and have been married 16 years. I just had a double lung transplant a year and a half ago. The Ichthyosis doctors said it is not due to my skin. My lung doctors thinks it might be related to my skin. I also had eyelid surgery on my left eyelids due to the eyelids being pulled back.

If you have any questions for me or my mom feel free to email me or I am on facebook Traci Peters-Snyder. snyders27@yahoo.com

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#28825 - 02/26/13 12:20 AM Re: Help!! [Re: Hearsay]
imranym Offline
Member

Registered: 02/23/13
Posts: 5
Thanks Jennifer. I thought so too that Doctor 1 seemed to most knowledge of the lot. We do have a follow up visit with him end of March.

Is there a group page on facebook that i should look for?

Thanks

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#28826 - 02/26/13 12:24 AM Re: Help!! [Re: Traci]
imranym Offline
Member

Registered: 02/23/13
Posts: 5
Thanks Traci for your help. Any idea on what age scale formation actually started?

So the condition improves with age - any ideas on how much it improves by as one grows?

Thanks

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#28833 - 03/02/13 05:04 AM Re: Help!! [Re: imranym]
imranym Offline
Member

Registered: 02/23/13
Posts: 5
Would anyone be able to share LI pictures of treated and untreated skins. The Baby's skin is turning dark all over

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#28834 - 03/02/13 05:48 AM Re: Help!! [Re: imranym]
Hearsay Offline


Member

Registered: 01/16/04
Posts: 1449
Loc: Richmond, VA, USA
Have you looked into Facebook yet? I haven't seen you there. There are loads of pictures on some of the groups. Look for "Friends of Ichthyosis and Ichthyosis Mommy Spot. There's also firstskinfoundation.org where you can email the staff and see if they can put you in touch with anyone close by.
_________________________
Jennifer
Ichthyosis-en-Confetti Type 2
Husband, Nathan - 10, Elliot - 7, Oliver - 4, all affected.
I also have an unaffected daughter, age 8.


email: jennifer at confettiskin dot com
facebook - find me on "ichthyosis mommy spot" or "friends of ichthyosis"

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