Keratosis Pilaris Atrophicans Faciei (Ulerythema Ophryogenes)

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Originally posted by Anne

Hi, I have had keratosis Pilaris on my cheeks since age 13. My cheeks are now quite scarred and worm eaten.(known as atrophaderma vermiculata) The other area effect is my eyebrows. These are also red with hair missing off the outer third. I was correctly diagnosed 5 years ago (at the age of 35) Since then I have been trying to find out information and treatments for this condition. I would like to share with this forum the things that have helped me and also put some questions to anyone who may read this who has this disorder.
1) Age - in my case the condition was it's worst in my teens, with my skin being red and raw and flaring up if exposed to heat or cold. I am now 40 and my skin is a lot more stable. Improvements probably started around the early 30's.
2) Pulse dyed laser treatment - there are a few articles on the net about this. I had this treatment done about 8 months ago. I was pleased with the results and noticed an improvement in the redness of about 40%. They recommend further treatments however at a cost of $2,000 (NZ) plus a long plane flight (only one person in NZ does the treatment) I have not ventured to have anymore. I would recommend that anyone suffering from this redness look into this because as far as I am aware this is the only proven treatment show in medical journal articles.
3) Retinova - this is a retina A cream. I found it improves the texture of my skin. I also use it because I am now 40 I am aware that it also helps keep those wrinkles at bay!

Questions I would like to pose to anyone with this condition are:
1) do you have any other congenital defects in your family (esp. those involving the ectodermal areas i.e. brain, teeth, bones and skin)I ask this as I have developed epilepsy for no known reason in the last few years and I also have a son with a congential heart defect. I would be interested to know if anyone else has a similar experience
2) Does anyone who has had keratosis pilaris on the cheeks resulting in scarring experience an aching type nerve pain in the effected areas and also around the eyebrows. With me this aching type pain developed in my thirtys and has slowly worsened. Doctors have labeled it purely psychosomatic - which it may well be! - howver no-one has been willing to look at it further as it is not noted in any literature. Any help in this area would be greatly appreciated as I am desparate to do something about this pain as it is effecting my life quite a lot. Any opinions most welcome.
Please also contact me here or direct on gogorman@xtra.co.nz if you want to ask me anything. Thanks for reading this long message!!!

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Originally posted by Tessa

Anne

I'm so glad you posted this, because you are the first person (apart from myself) that I have heard of who suffers from this variety of KP. Please see my earlier posts regarding it.

I suffer from this on my forehead. Like you I was not diagnosed until my thirties. I believed before this that I had mild acne in this area and that the strange 'open pore' markings were either enlarged pores or acne scarring. I was given retin-A to deal with it, but it has caused increased redness and irritation and I have simply stopped using it.

I'm still not really sure how to deal with it. The laser treatment sounds good.

As for other congenital problems in the family - there is a lot of quite severe eczema in my family, and as KP is associated with dryness, I imagine that my condition is connected to the same set of genes that causes the eczema. At the moment, I am experimenting with an Osmotics cream designed to 'repair' the barrier layer of the skin (to help with the dryness and flaking) and will also use exfoliating lotions a couple of times a week (I use neutrogena AHA and Paula's Choice BHA).

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Originally posted by Anne

Thanks for your response Tessa. I have been in contact with a few people via email who have keratosis pilaris atrophicans faciei and there seems to be slight variations. Some just have it around the eyebrows others have scarring and loss of hair in the scalp area. As mentioned I have
pitted scarring on both cheeks. The red bumps
have gone with age except for a few around the eyebrows where the hair has been lost. I have been encouraged though with the develops in the area of cosmetic surgery. 25 years ago when the condition first appeared on my skin the only treatment I had to help my red raw cheeks was to have them burnt with liquid nitrogen. I wouldn't recommend it to anyone. Having a board like this is really positive as people can post details of treatments they have had that have helped. When one has a rare disorder like Keratosis Pilaris Atrophicans Faciei it is frustrating trying to find information and help. Keep looking and let me know if you find anything that helps you.

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Originally posted by Tessa

Anne, I can really relate to the fact that when you have a rare disorder, there is very little information available - unlike say eczema or rosacea.

I think my disorder is only really noticeable 'close up' - many friends I have mentioned it to have been genuinely surprised that there is something wrong with it. As regards harsh treatment, I think that I may have perhaps 'induced' the KP on my forehead with harsh acne treatments. Absolutely no one else has facial KP in my family, and I don't have it anywhere else on my body (except a few on the back of the arms which come and go). My facial skin is often flaky around my eyebrows and nose and I seem to get dermatitis like symptoms on the forehead and around the nose. I have had no hair loss. I'm just trying to treat it as gently as possible for the time being.

As this variety is so rare, I wonder if we share a common gene that triggers this disorder off. I am originally from the UK (now resident in the US), where are you based?

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Originally posted by Anne

Thanks for your response Tessa. I have been in contact with a few people via email who have keratosis pilaris atrophicans faciei and there seems to be slight variations. Some just have it around the eyebrows others have scarring and loss of hair in the scalp area. As mentioned I have
pitted scarring on both cheeks. The red bumps
have gone with age except for a few around the eyebrows where the hair has been lost. I have been encouraged though with the develops in the area of cosmetic surgery. 25 years ago when the condition first appeared on my skin the only treatment I had to help my red raw cheeks was to have them burnt with liquid nitrogen. I wouldn't recommend it to anyone. Having a board like this is really positive as people can post details of treatments they have had that have helped. When one has a rare disorder like Keratosis Pilaris Atrophicans Faciei it is frustrating trying to find information and help. Keep looking and let me know if you find anything that helps you.

[Archive]

Originally posted by Anne

Thanks for your response Tessa. I have been in contact with a few people via email who have keratosis pilaris atrophicans faciei and there seems to be slight variations. Some just have it around the eyebrows others have scarring and loss of hair in the scalp area. As mentioned I have
pitted scarring on both cheeks. The red bumps
have gone with age except for a few around the eyebrows where the hair has been lost. I have been encouraged though with the develops in the area of cosmetic surgery. 25 years ago when the condition first appeared on my skin the only treatment I had to help my red raw cheeks was to have them burnt with liquid nitrogen. I wouldn't recommend it to anyone. Having a board like this is really positive as people can post details of treatments they have had that have helped. When one has a rare disorder like Keratosis Pilaris Atrophicans Faciei it is frustrating trying to find information and help. Keep looking and let me know if you find anything that helps you.

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Originally posted by John

Hi Anne,
you wrote that your son has heart problems.
Did you ever think about the noonan syndrome that is often related to KPARF?

I also have Ulerythema Ophyrogenes and tried various lasers.If you want to go on with the laser treatment then you only should be treated with the longpulsed dye laser.I had over 10 treatments with the old dye laser and had only less 30% improvement! The new one works much better and faster and also helps for flushing not only for normal redness!
Stop the topical steroid treatment.It helps in the beginning but after few month your cheeks get more and more red!!Then it makes the atrophy much worse.
BTW i think many people in this forum overrate their normal KP.They should only live few days with KPARF then they might be happy to only suffer normal KP!
All the best to you and your child!

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Originally posted by Anne

thanks for the information John. Can you give me the full name on the 'long pulsed dyed laser' or a reference to an article about it. As I live in NZ treatment is not always available here but here I have some specific info I could ask around. As far as my youngest son goes - yes I have looked at Noonan's but fortunately he is very bright and healthy. He had transposition of the great arteries which was correct at birth plus a small defect on one of his valves. Their is obviously a genetic link between KPAF and the heart - don't ask me what though! Yes KPAF and ulerythema ophryogenes certainly make life a lot more challenging. One has to work hard at keeping ones self esteem up. Most days I say to myself a verse from the bible - it says "you are fearfully and wonderfully made". God doesn't make mistakes and I believe having this disorder has worked a lot of good things into my character that I would not have possessed if life had been a bit more cruisy.