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What is the prognosis for epidermal nevus syndrome?

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Practicing since : 2005
Answered : 3360 Questions
Hello....this question pertains to my nineteen year old son and a pigmented lesion he has on his upper back. Unfortunately, I'm suffering from some health anxiety and made the mistake of googling what his dermatologist diagnosed as a "linear epidermal nevus", and I ran across information pertaining to some associated syndromes and genetic mutations that could be passed to offspring.

My son has had a small lesion on his upper back which I first noticed when he was about ten years was slightly raised and light brown and resembled a cafe at lait mark; I had showed it to his pediatrician through the years, who expressed no concern.

My son is now 19 years old and has no diagnosed medical conditions. The lesion is still on his back, is about 3/4 of an inch and has become slightly darker and rougher through his puberty. It resembles three smaller lesions closely grouped together. A few months ago I took him to the dermatologist, who examined it with a dermascope, and diagnosed it as a solitary "linear epidermal nevus". She indicated that it has zero to little chance of becoming dangerous and they would just monitor it yearly.

Again, I made the mistake of googling linear epidermal nevus and ran across information relating to epidermal nevus syndrome and information indicating that a certain type of the lesion (epidermokytic?) could be caused by a mutation in the keratin 1 or 10 gene(?) and future offspring could have some serious scaling skin disease. My son has only the single solitary small epidermal nevus and no other systemic diseases.

I realize this question is quite "neurotic", but do you feel there is anything to worry about (as a neurotic mom) with regard to that syndrome or the risk of genetic mutations passed from him to future offspring? I presume such a probability would be very rare, as opposed to something like the gene for cystic fibrosis, which I understand is much more common.

Thanks so much for any reassurance you can give to me with this obscure question!!
Posted Wed, 22 Nov 2017 in Genetics
Answered by Dr. Diptanshu Das 4 hours later
Brief Answer:
Please do not get neurotic about this

Detailed Answer:
Thanks for asking on HealthcareMagic.

I have gone carefully through your query and understand your concerns. "Linear epidermal nevus" is otherwise a harmless condition but since it is a neurocutaneous disorder, it can have neurological manifestations. It is a very rare condition. Mutations in the HRAS gene is usually found to be associated. Depending on the variant there can be a few other genes involved as well. Although transmission to offsprings is a possibility, I do not suppose that it makes any sense to worry about it. It is no use worrying about things that may or may not happen and there is nothing that you can do about it anyway. I would therefore insist you not to panic. This is not a life threatening condition and so, even if the genes get passed on, it would not be life threatening in that case too.

I do not think that you should try and ascertain which specific gene is involved in the case of your child.

Let me know if I could help further.

Above answer was peer-reviewed by
Follow-up: What is the prognosis for epidermal nevus syndrome? 44 minutes later
Hi Dr. Das! Thank you so much for your comforting words and for putting it into perspective. Unfortunately, these rare and unusual conditions that invariably end up coming through in a google search can wreak havoc on those of us who suffer from health anxiety! Hearing a more realistic view from a real life doctor is so comforting and reassuring!

So just to make sure I'm clear, would you say that my son having a singular and small linear epidermal nevus is not an unusual occurrence, and the fact that he has no other health conditions at age 19 points to him not having that related syndrome? Also, when you indicated it's very rare, was it the epidermal nevus syndrome and gene mutation you were referring to, and not the existence of a single nevus?

Thank you again immensely for your kindness and understanding with my google-induced neurotic questions!!
Answered by Dr. Diptanshu Das 54 minutes later
Brief Answer:
No need to worry

Detailed Answer:
I would request you to upload an image of the linear epidermal nevus. A visual confirmation will help me to correlate.

A linear epidermal nevus is relatively rare but that does not mean that it is something alarming. Since your child does not have other neurological manifestations like seizures, I do not think that you should bother about it. It does not really matter whether it is a part of a syndrome (a categorisation of ailments done by clustering a common set of symptoms) and whether it is caused due to an underlying gene mutation. We do not try to explain the embyological origins of everything about our body. It is rather incidental that we happen to have an understanding of how things proceed embryologically in case of such a nevus. At your level, forget this extra knowledge and just go about your regular life. You have no cause to worry.

Above answer was peer-reviewed by
Follow-up: What is the prognosis for epidermal nevus syndrome? 27 minutes later
That is a wonderful answer Dr. Das! Thank you so much for putting into realistic perspective this information I unfortunately stumbled upon by googling!

I would love to send you an image of the nevus, however, I simply do not want to ask my son to let me take a picture of it. The dermatologist we visited seemed to be confident in the diagnosis and not concerned about it (as I should be!), and I don't want my son to think that I'm doubting that diagnosis or worried over it!

You have been so very helpful and understanding with your expertise and compassion, and I cannot thank you enough for your kindness!!!
Answered by Dr. Diptanshu Das 36 minutes later
Brief Answer:
Glad to be of service

Detailed Answer:
I am glad that my words have been reassuring to you. It is not that I doubt the diagnosis. It is that perhaps by having a look I can throw in a few additional bits of information that can be of use to you. I am a bit inquisitive about the lesion and what made the doctor arrive at such diagnosis. Most often a dermatologist simply refers to such a lesion as a simple nevus without any syndromic association. That is the reason for me wishing to have a look.

Feel free to ask me back in case you have any further health related queries in the future.

Above answer was peer-reviewed by
Follow-up: What is the prognosis for epidermal nevus syndrome? 12 hours later
I am so pleased with Dr. XXXXXXX and his kind, informative, compassionate and reassuring response! He allayed my fears and is a very very special physician!! Many thanks!
Answered by Dr. Diptanshu Das 7 hours later
Brief Answer:
Glad to be of service

Detailed Answer:
I am glad that it helped. I thought that you would attach the image but did not find it attached.

Thanks for the positive review but it needs to be put in the appropriate section and not as a follow up.

Above answer was peer-reviewed by
Follow-up: What is the prognosis for epidermal nevus syndrome? 18 minutes later
Oh I'm sorry about that Dr. Das! I'll be sure to follow up with posting my review in the appropriate section!

I'm sorry I didn't attach an image, it's simply that I do not want to ask my son to let me take a picture, as I do not want to alarm him by thinking I'm still worried about it.

As long as as I can still follow up with you (I thought I had closed and submitted my review)...can I run something by you? I shouldn't have done this, but I googled a bit more and saw images of a "Becker's Nevus" and am now wondering if my son's lesion is more likely to be that type of epidermal growth (again I'm sorry to not attach an image to help). He was not born with it, and it did not develop until he was about 12 years old, just starting through puberty. As he's gone through puberty it has changed from a flat light brown spot to being darker and a bit rougher; it's about three quarters of an inch in size and it located at the upper back of his left shoulder. Come to think of it, his paternal grandfather (who is 85) has a very similar and larger lesion on the back of his left shoulder.

Given the location and time of development of it, it seems to make more sense (and no, I promise I won't panic over Becker's nevus syndrome!!)
Answered by Dr. Diptanshu Das 8 hours later
Brief Answer:
Becker's nevus more likely

Detailed Answer:
Sorry for the inadvertent delay in responding. Linear nevus syndrome is usually of early onset. The very fact that it was late onset rather points towards Becker's nevus. Since genetic factors are involved, it is not unlikely that there is a connection with that of his grandfather's.

I am glad that you are no longer panicking.

Above answer was peer-reviewed by
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