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New IP diagnosis
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New IP diagnosis

We just had our first baby and she was diagnosed with IP She was born on 09/22. She's a lovely girl, and we really feel blessed to have her. We had a normal pregnancy, however, when she was born she had a few blisters on her body who were thought to be pustular melanosis. On the second day of life,  she developed a warty angry rash all over her body. She was doing well otherwise, but we were concerned about this so we consulted a dermatologist who immediately said she has IP. By the fourth day of life the rash was almost gone, and in some places where she had it there were freckles that followed a similar pattern to the rash.  After much reading, we realize we have been lucky to have a diagnosis so quickly. Neurologically , she is doing fine it seems, but because of the extent of skin involvement, we had been referred to a neurologist and an ophthalmologist.
When we first heard about her diagnosis, we were emotionally devastated. After educating ourselves on the disease, we feel that there is hope for our daughter to live a normal life. As far as we are concerned we just care for her to have no neurological problems. We have decided to support her and do as much as we can for her.  We would like to connect with other parents who are going through this, and also, discuss our concerns with people that are in similar situations to ours.  At this point we have a few questions that no one has been able to clarify and that I hope that based on your personal experience (each of you) would probably be able to answer.
1) Have you ever seen the warty rash disappear so quickly? from what we have read this takes months to years and in the case of our daughter it took literally 3 days.
2) Does the duration of the rash bear any prognostic significance as far as her brain/neurological status?
3) Does the amount of skin involvement correlate with prognosis and extension of disease (meaning systemic involvement)
4) How often is that neurologic involvement is seen? does this correlate with anything else? (e.g. eye involvement, etc).
5) I also read that there is a correlation between IP and being immunologically compromised. If this is the case, what precautions does one need to take to keep the baby as healthy has possible?

Thanks!
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17 Comments Post a Comment
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620222_tn?1294434326
Hi there! I can't even tell you how familiar your story is to me..and your feelings! My daughter Rachael is 15months old now and is doing great.  Like your daughter she had a few little blisters at birth that went away, and then at 3 days old she broke out with terrible blisters all over her arms, legs, bum & scalp. The first pediatrican we saw when she was 3 days old thought too that she had pustular melanosis but it should have cleared up by the time she was a week old and it hadn't.  Plus her blisters weren't leaving behind the charachteristic brown "freckles" assosiated with (transient neonatal) pustular melanosis.  She had terrible blisters/warty lesions for about 8-10 weeks.  They would almost clear up and then come back...over and over.  Are you having your daughter tested for IP to confirm the dx through Baylor at all? We had Rachael tested and confirmed IP through gentics testing there.  I was also tested and am not a carrier so she is a sponatenous mutation.  where are you from?
not sure about your other questions but you could contact the IPIF (.org) and she might be able to help you?
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Avatar_m_tn
hey, thanks for replying. It's been a while..... our daughter has been seen by an ophtalmologist and a neurologist. She has been ordered an MRI/MRA just to evaluate for anatomical problems. She had some blood drawn, but I am not sure if it will be sent to baylor for analysis (I am assuming it will, since it seems to be the only place in the country where they can detect the gene).  She is doing really well it seems, she has met all of the milestones for her age (now 2 months) and she has a few lesions left which are warty like, mainly one of her legs and one of her hands. She does have a lot of "freckles" in all of the areas where she had the rash.  
I tried contacting the IPIF but I did not hear back from them. We live in southwestern PA....... what about you?
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620222_tn?1294434326
we live in Paris,ON (Canada) --its in between toronto and london--so not too far from you guys I guess? Maybe about 6 hours drive?
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658757_tn?1397800478
ip is one of 150 rare genetic disorders that falls under ecodermal dysplaysias. if you go to this website it may give you more info. www.nfed.org. my daughter has ip as well, but was born with group b strep, because i didn't get the anibiotics in time. she has the skin pigment, misshapen teeth, neurological problems, development delayed. 2 lesions on the left side of her brain, due to group b strep. she had sezuires since birth, but went undiagnoised until she was 3 months. what i have read up on, on this website, is that the skin pigment goes away as they get older. if you have any questions at all about anything, please let me know and i will try to help the best i can.
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Avatar_f_tn
Hi there. I too have a daughter with IP. Diagnosed when the was 4 mon and is now 7 mon. She seems to be meeting all her milestones. The only concern I have is she hasnt cut any teeth yet. We took her to the derm dept at CHOP and due to insurance issues are taking her to Mount Sinai in NYC. I can relate to the uneasy feeling of what the future holds. Everything I have read and professionals I have talked to seem very upbeat about a positive future for her.
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546640_tn?1267062157
Hi, i just came across your posting, i too have a daughter with IP, she is 2 years old now and luckily she is doing great. She was born fine, when we brought her home, she had a seizure and another one so we brought her back to the hospital, they did many many tests and finally gave me the diagnosis as IP. she had really bad rashes and blisters and could not drink well out of a bottle. we had to teach her, it was tuff, we were really really scared because before the diagnosis, we didnt know at all what she had. we were in the NICU for another 10 days but finally she got better, learned how to drink and was put on anti seizure medication, ( phenobarbitol ) she was on it for 9 months, then the neurologist weened her off slowly over 6 weeks. she did not get a tooth until she was 1 she has about 6 teeth now, i would say 3 are normal and 3 are really tiny, they are scattered and i know she will not have any on 1 side of her mouth. but i feel that that is basically cosmetic and could be taken care of. She has been developing normally, i did have to follow up with eye doctors, dermatologists, nuerologist , dentists, and her regulary doctor but i have to say... she is one of the lucky ones, and us too, because her issues are very slight. i just thank god that her brain is completely perfect and i pray that she will have a long healthy fulfilling life. Please write to me, i would love to keep in touch.  i have to update our profile and upload some new pictures. good luck and god bless your baby
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Avatar_f_tn
Hi every one, My daughter had IP as well, She developed the rashed when she was 3 days old and stright away the doctors diagnosed her. She is 11 months now and doesnt have any tooth yet. She is very good. We take her for eye checkup every 3 months, so far she has been good. I wish all our baby grow up healthy. We live in Sydney Australia. The doctors here havent seen much Ip patients.
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Avatar_n_tn
hay, I have a daughter wit IP. She is 10 months old now and she is doing all great. She has skin problems, she was lasered twice (right eye). She has 2 teeth which seem to be normal. Is there anyone who has a baby girl with normal teeth? Is it possible for the baby with IP to have normal teeth?
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Avatar_m_tn
hello. Our daughter is 7 months old and it seems that her teeth are coming out. We can see white on her gums and it seems that she will have at least the upper teeth. It's too early to say and these are decidual teeth, so we don't know what the future holds. We have been asked to see a pediatric dentist so she can be further evaluated and see if there is anything that can be done regarding her teeth. If I recall correctly 90% of the children with IP have teeth abnormalities of some kind, this means that there are 1 out every 10 that do well......... yours might be one of them.
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Avatar_m_tn
Sara, so nice to hear your baby is doing well. We thank you for your good wishes!
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Avatar_m_tn
it seems that your baby has done marvelously despite the seizures she had at the beginning, I am very happy to know that she is doing so well. We wish you the best and I hope to see more pictures of her!  
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Avatar_f_tn
I have IP and I passed it to my daughter. She is 6 months old now and reaching all of her milestones. While there is not too much information on this it all seems so scary! Just letting everyone know I lead a very normal life! While I still have scars they are very light and you really can't even tell! My teeth were horrible but I got them capped- It is important however to get your children to the dentist very early on..... I was missing teeth and it made my bone structure of my cheek different. I also have to wear contacts but no real eye issues. If I can help anyone please feel free to email me @ ***@****
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Avatar_f_tn
my daughters main problems have been with the underdeveloped jaw and missing teeth.  She has no bottom teeth on 1 side and several missing teeth scattered throughout.  I have been to 2 dentists who say they can not attach any appliance to her teeth to fill the gap because she has nothing to attach it too.  I am going to a new dentist Monday and hopefully they can help her.  She calls 1 side of her mouth her drinking side and the other the eating side.  Not that she eats much, it has always been a fight.  I feel blessed that she has not developed any other problems.  She is now 7 so hopefully with prayer that will continue.
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Avatar_m_tn
hey, I hope you have found a dentist to take care of your daughter. I think the best option you have is to have her be seen at an academic center (ie big teaching hospital) they tend to have the most resources. Also if you browse the IP association website there are some resources for dentists and places that will subsidize the dental work. Give us an update on your case.......... hope you are doing well!
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Avatar_f_tn
Hi, does your daughter have the skin with the brown swirls and somewhat spotted?
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Avatar_m_tn
Hello,
yes she does, mainly on her legs and chest. She has 2 missing desidual  (baby) teeth but otherwise she's a  perfectly normal 3 year old!
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Avatar_m_tn
hello- just wanted to see how has she done? any luck? did you find a dentist that help you?
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