neurogenic rosacea -- my battle.

[laser_cat]

Hello

So … for the past 2 years I’ve been struggling with neurogenic rosacea (I’m 32/f) I felt so broken for a long time. I could not work because of the burning pain or leave my temp-controlled home, etc. I just want to say to anyone out there struggling like me, to not give up, to make sure you are trying at least *something* at any given time, be relentless in reading about this condition from forums, researching appropriate doctors, but especially going through medical literature on potential pathophysiology, the way potentially useful drugs work, and about related conditions (primarily erythromelalgia, but also migraine, autonomic dysfunction, diabetic neuropathy and other types of neuropathy, raynaud’s, vasospasm, burning mouth syndrome, non allergic rhinitis, red ear syndrome, etc).

I am fortunate to be near 2 major teaching hospitals. After a year of bouncing around referrals, I finally had an appt with one of the docs who came up with the paper designating “neurogenic rosacea” as a separate disease entity. I also am very fortunate as well to see world-class doctors in pain management, rheumatology, neurology, and now that I can see the light at the end of the tunnel, I couldn’t in good faith not pass along my experience + what I’ve learned + speculate with all this for someone out there like me who might be struggling.

my symptoms — burning + flushing every night ~ 6pm-2am, that would come out of nowhere. At first I’d be ok during the day, but after ~6 months it became all the time. I would pace my room at night because I couldn’t sit down or lie down to sleep. Thinking hard about a problem, talking to anyone, chewing, digesting were not smiles times. Laser - not helpful in the least.

[laser_cat]

The first big med I tried was clonidine. I thought it was very helpful at first. But maybe only a week (?) and my body got used to it perhaps, and it was doing more harm than good. I only know this in hindsight. Taking a dose would help to clamp down the current flushing, but it was setting me up in the long term for more frequent and painful flushing. Another thing I’ve noticed — it worsened my flushing due to sympathetic nervous system spikes (like talking to anyone). Stayed on the drug for over a year before I gradually tapered off. On clonidine, my skin (including face) could get cold to the touch, so with every temperature increase, it felt like my body had to “save” the skin (I do not and never had obvious Raynaud’s, but perhaps tendencies). I found it to be a very powerful vasoconstrictor. It works as being a central alpha 2 adrenergic agonist (amongst other things). I haven’t confirmed this with doctors, but I believe it can backfire in a way that is reminiscent of Mrivaso (even though clonidine is centrally acting — meaning CNS — and Mirvaso is peripherally acting). To me an “agonist” is a dangerous mechanism of action (your body might over time down-regulate those receptors that are being activated) as compared to an “antagonist” mechanism (like a beta blocker, which block the epinephrine from normally causing peripheral vasodilation). If your body adjusts to the beta blocker presumably by making more beta 2 adrenergic receptors in peripheral blood vessels, and you need more benefit, you can just up your dose. But back to clonidine - I believe it was obscuring the results of testing out other drugs because it was such a dominant vasoconstrictor. I basically had to try all the drugs that I had tried while on clonidine, a second time, and some things that weren’t successful on clonidine, became successful (see mexiletine). My 2 cents is - if you’re on it and it’s still not enough of a relief for you, to get off of it at least temporarily (taper off very slowly) and try other meds before going back.

[laser_cat]

I think that a mechanism that could be at play for some people is AV shunting. It is thought to occur in complex regional pain syndrome, erythromelalgia (EM), sometimes Raynaud’s, diabetic neuropathy .. and I have yet to read a reason why this couldn’t happen in the face. It’s when the blood never quite reaches the skin, but is shunted / short circuited away from the capillaries, paradoxically causing hypoxic skin tissue even though the body part affected can be hot/swollen/flushing/burning.

Another mechanism that could be at play for people is vasospasm (like Raynaud’s or migraine). The pain comes from having blood vessels that are too overly constricted at a basal state, so when there is a temp bump, the dilation is excessive and very painful. (Vasospasm and AV shunting may not be distinct mechanisms.)

[laser_cat]

My doctor who came up with the neurogenic rosacea subtype and diagnosed me as such — I asked him how it is different from erythromelalgia (EM) many a time. He says “neurogenic rosacea” is just a name they came up with, but that when he thinks of my case, he puts it in the category of traditional EM involving feet only. He’s seen cases of someone with just a burning face which then progresses to hands/feet. According to him, he thinks everything *starts* with sensory nerve dysfunction (as opposed to thinking about it like a vasospasm). This is why stellate ganglion blocks, purely vasoactive drugs, SSRI’s will only go so far with cases like mine. The success of SSRI’s or beta blockers— which I was hoping at one point to calm down my overactive sympathetic nervous system — assume normal sensory nerve function which he says I do not have. He speculates that by addressing the discomfort, the flushing component will naturally recede. He is the only doc so far to push the agents blocking sodium channels (which are thought to be key in EM and other chronic pain conditions). Lidocaine IV’s are one of the few agents to uniquely address abnormally functioning sodium channels — getting them done regularly might be able to reset the pain neural circuits at play and uniquely address the root of the problem. Mexiletine and ranexa (there are a few others like oxtellar, lamotrigine, valproic acid, phenytoin that also address sodium channels) are oral sodium channel blockers but without the same power as lidocaine. It’s possible lidocaine iv might "fast-track" a response to mexiletine for example. Hyper excitable sodium channels is thought to recruit sympathetic involvement and contribute to flushing and redness. While some people get one lidocaine iv as outpatient and are good to go for the next couple months, I am told that this is not the norm and others need longer / more frequent lidocaine to retrain the pain circuits.

Dry skin that cannot sweat can be from an underlying neuropathy. I was given neurodiagnostic testing (tilt, sweat, etc) and was found to have small fiber neuropathy (coexists a lot with EM) and autonomic dysfunction, which seems to support the hypothesis of nerve dysfunction being the root in all this. Currently my neurologist is going to try to figure out the cause of the neuropathy and see if it can be addressed. He has mentioned some instances of inflammatory neuropathies that can be treated with IVIG and high dose steroids for example. Many EM patients (50-90%) also have small fiber neuropathy, and can show a decrease in epidermal nerve fiber density with biopsy. As the nerves try to regrow, the pain circuits might get out of control (my understanding). Continually icing the skin can also lead to small fiber neuropathy and perpetuate the pain. Unfortunately no one can take biopsies to measure nerve density in the face or ear areas - because it is not standardized there yet (no "normal" data to compare it to). But sometimes low nerve fiber density can show up in other areas where it is standardized - along the leg or arm. A derm, rheum, or neuro can take the biopsy and send it to a neuropathology lab for analysis like Therapath or Corinthian Reference Lab.

[laser_cat]

It took years for doctors to take me seriously and not just shrug and say “maybe it’s stress?” Unfortunately I found it necessary for a close friend or family member to come with me to doctor appts so my experience can be validated by someone who isn’t me, at how much this has taken over my life, and that we cannot just hope it would just “go away”. I think severe flushing / burning faces might be on the cutting edge of things in the medical community (for better or worse), and it’s imperative to find a doctor who can contemplate different hypothesis from similar conditions, pivot with new information as every case is a little different, and is aware of the assumptions s/he is bringing to the table (e.g. does it start with the blood vessels? does it start with the nerves? am I even aware I am having this assumption so I can backtrack if necessary?) when in fact nothing is *really* known yet. I definitely wouldn’t trust just *one* doctor either — having a team of doctors can bring unique perspectives to the table. In particular I wish I had gotten a referral into the pain management center much sooner — they are one of the few who recognize that chronic pain needs to be treated regardless of the cause because it can quickly become a life force of its own (and this is my experience as well). It is usually pain doctors who offer lidocaine iv’s (not derms).

I know this is a novel but I actually have a lot more I could say and was really trying to pare down believe it or not :p Good luck to everyone.

[rednessator]

Thank you very much for sharing, very interesting read, and will help somebody for sure! Its obvious you been through a lot, from the bottom of my heart, keep fighting, glad you are finally seeing the light!

[laser_cat]

Thank you very much for sharing, very interesting read, and will help somebody for sure! Its obvious you been through a lot, from the bottom of my heart, keep fighting, glad you are finally seeing the light!

Thank you rednessator, means a lot Wishing you success as well xx

[MissM]

Hi Lizzy!

Thank you so much for thoroughly documenting your experience. All this information is extremely helpful and it's a great resource and reference.

One question: do you still think that coming off the birth control pill is related to the neuropathy? What do your doctors say about this?

M.

[laser_cat]

Hi Lizzy!

Thank you so much for thoroughly documenting your experience. All this information is extremely helpful and it's a great resource and reference.

One question: do you still think that coming off the birth control pill is related to the neuropathy? What do your doctors say about this?

M.

Hey MissM!

Honestly I don't know. My hunch is that coming off the birth control pill was not the cause at all though. For a while I was really zoomed in on that, I know. Around the time of my flushing/burning onset, I also had migraines (why I came off the pill). Ive talked to a couple other people with facial burning who say their migraines were in peak form around their facial burning onset. So maybe it was more about my migraines than coming off the pill ..

No doctor knows of any connection between birth control and all this, either in the medical literature or in their experience.

So anyone who's on the pill and is thinking of coming off ... I think you'll be ok

Best,

[MissM]

Hey MissM!

Honestly I don't know. My hunch is that coming off the birth control pill was not the cause at all though. For a while I was really zoomed in on that, I know. Around the time of my flushing/burning onset, I also had migraines (why I came off the pill). Ive talked to a couple other people with facial burning who say their migraines were in peak form around their facial burning onset. So maybe it was more about my migraines than coming off the pill ..

No doctor knows of any connection between birth control and all this, either in the medical literature or in their experience.

So anyone who's on the pill and is thinking of coming off ... I think you'll be ok

Best,

Thank you, my friend! <3

[sg321]

Lizzy,

Such awesome news! Yay!! Congratulations on winning this battle. Perseverance certainly pays off.

Is the Amlodipine helpful for pain and flushing? It’s so interesting to me how a drug that would apparently worsen rosacea at higher doses could be so helpful in a smaller dose. Did it help with permanent redness at all?

Thank you,
SG

[laser_cat]

Lizzy,

Such awesome news! Yay!! Congratulations on winning this battle. Perseverance certainly pays off.

Is the Amlodipine helpful for pain and flushing? It’s so interesting to me how a drug that would apparently worsen rosacea at higher doses could be so helpful in a smaller dose. Did it help with permanent redness at all?

Thank you,
SG

Hey SG -

Thank you so much. The battle is not won yet for sure but at least I feel like I'm on the right track I am very lucky to have supportive family and access to good healthcare, otherwise I'd be nowhere for sure.

The amlodipine is helpful for both pain + flushing, I think by evening out blood flow / oxygen tension in my face. It is esp. helpful for the evening burning -- how my facial skin can be BOTH softer and not as hot, seems a bit couterintuitive! I had to come off it for my neurodiagnostic testing and the burning just came right back. It is neutral for my baseline redness, albeit caused flushing the first time I took it.

Yea, the lowest pill comes in 2.5 mg and I take about a quarter of that only lol :p (Actually I now have the liquid form for baby dosing.) There was an informal poll amongst EM patients regarding magnesium (a natural calcium channel blocker) -- about a third get better, a third have no result, and a third get worse. (I had tried mag with no benefit). Sucks how much this is trial and error!

eg - https://www.ncbi.nlm.nih.gov/pubmed/11847944

"These results suggest a possible role for high-dose oral magnesium in the treatment of EM and, perhaps, other vascular disorders."

Wishing you well,

Lizzy

edit - amlodipine isn't really effective for me anymore, but might be for others.

[laser_cat]

The drugs I was rx'ed by my very good derm (in this order):

gabapentin
lyrica
doxepin
(already on cymbalta, but he would have rx'ed it)
mexiletine
plaquenil
ranexa
nadolol
topical amitriptyine / ketamine (I think all medicated topicals have potential to sensitize/irritate face skin)
encouraged lidocaine iv's
encouraged IVIG (1 gm / kg / mo)
marinol (marijuana THC)
baclofen
retry IVIG at higher dose / longer time frame (2 gm IVIG / kg body weight / mo for 6 mo)
azathioprine

by pain doc:

lidocaine iv
ketamine iv
butrans
keppra
trileptal
gamma core (tVNS) (This is good in theory but I think the vibration on neck made me worse)
Aimovig (in hindsight, I do not see how this can be effective)
topical diazepam
topical baclofen
stellate ganglion block
trigeminal block
zonisamide
flecainide

by another derm:

midodrine (in hindsight, I do not think a good choice)
low dose naltrexone
indomethacin (in hindsight, I do not think a good choice)
high dose antihistamine (2x zyrtec + 2x allegra)
botox (in hindsight, I do not think a good choice for severe cases)
amlodipine
propranolol ER
aprepitant (could not get insurance coverage more than 2 days, very difficult to do so)
tegretol
offered butorphanol nasal spray
topical amitriptyline
topical bupivicaine / lidocaine / tetracaine
memantine
glycopyrrolate (in hindsight, I do not see how this can be effective)
suggested topical capsaicin OTC 5x/day, until acclimation, if I wanted to give it a go (warned me he never had a patient use on face/ears)
daily triptan (frovatriptan)
oral metronidazole (just to get a handle on edema) - later switched to antibiotic Keflex (also offered Azithromycin)
offered tramadol
Xeljanz
Tanezumab (compassionate use)


by neuro:

IVIG (1 gm IVIG / kg body weight / mo for 3 mo)

by other docs (PCP, psych,..):

clonazepam (I have never heard of this being effective "enough" for people)
mirtazapine
cymbalta vs. paxil vs. effexor
atarax
clonidine
topamax
gastrocrom (note: I'm told this doesn't reach skin, so I don't know how practical this is)

by another (very good) derm:

methotrexate + folic acid (I personally couldn't tolerate this)
Cellcept
high dose IVIG (2 gm / kg / mo)
Trental for erythromelalgia symptom management

*****

edit: In hindsight, I think aggressive nerve-based treatments interleaved with aggressive inflammation treatments is the most efficient trial/error algorithm.

[laser_cat]

.

[laser_cat]

But continuing with update stuff - My neurologist took punch biopsy in my leg and confirmed I had non-length dependent small fiber neuropathy. My legs are fine, but there can be a lower density of nerves even in “ok” regions in neuropathies. I wonder how many people with disabling flushing/burning on here + other groups, would end up having positive results, esp. women with acute onset, possibly with coexisting immune issues. Erythromelalgia is the term used to describe burning feet and often goes hand-in-hand with (length dependent) small fiber neuropathies (either dysfunctional blood vessels + maldistributed blood flow causing hypoxia and neuropathies, or the neuropathy causing dysfunctional blood vessels … the vasculature + nerve dysfunction often dovetail) Facial erythromelalgia is a controversial diagnosis and is generally not given. However, just as small fiber neuropathy doesn’t always start with the feet, I wonder why there is a mental hurdle in the medical community acknowledging that maybe, the same or at least similar phenomenon that we recognize as EM (which is not even a separate disease entity, but a symptom complex much like rosacea is, without any diagnostic test) might start in the face/ears/trunk? When I read papers by medical experts on EM and flushing (see later .. I included a couple back and forth letters on "facial erythromelalgia?"), it honestly sounds like a territorial pissing contest with a conspicuous omission of anything factual; I believe the unfortunate consequence of physicians shying away from "facial Erythromelalgia" diagnosis or at least consideration, is that appropriate treatment is often delayed.