Flushing is one of those things you most likely recognize fairly quickly when you have it, but to help define it, patients have described it as the feeling of having bad sunburn; like the skin on your face is many degrees too hot, glowing, burning, with heat crawling up. Radiating heat. Swelling, stinging. Some people would say that it feels like an iron being pressed to your face, and the heat coming from the inside out. Often the face turns pink or red as well, but this isn't the case for everyone. The skin can also feel tingling and itching, or numbing. The skin also tends to become very sensitive, to a point where even touching your face can be uncomfortable. Others feel like they are running a fever and are badly overheating. For many people with rosacea, being in the sun can be a trigger and make the redness and potential burning worse. A lot of people also notice that drinking alcohol can make their face more red. Spices and emotional stress can do the same. I think that what makes rosacea typical, is that it can make someone's skin go red in a matter of seconds or minutes, when triggered. This mostly goes for subtype 1, and is almost like a heavy blushing attack. But the difference between rosacea and blushing is that over time the 'blush' from rosacea takes quite a long time to subside. And these flushes become more frequent. And with time, many people also find that the redness of the skin starts to hurt, or feel hot. Sometimes the face even becomes a bit puffy. As I said, these are no strict criteria and rules, but maybe they can help you in the right direction. A dermatologist should be able to properly diagnose you ideally, because there are more skin conditions that can look like rosacea, including dermatitis, seborrheic dermatitis, acne or the skin redness might be linked to an allergy, or a systematic illness like lupus even. It's up to a dermatologist to make the right diagnosis. [Paintings and art works on this page are made by my partner].
-Seborrheic dermatitis/eczema
-Systemic mastocytosis (and other histamine intolerances)
-Erythromelgia (EM) is also a very rare cause of facial flushing
-Lupus erythematosus
-Polycythemia vera
-Mixed connective tissue disease
-Hormone imbalances/Menopause
-Drug related flushing
-Allergies/ Food allergies/intolerance/ coeliac disease
-Keratosis Pilaris Rubra faceii
-Carcinoid Syndrome/pheochromocytoma
-Thyroid problems
-Heliobacter infection/ SIBO
-Photosensitivity
-Harlequin Syndrome
-Auriculotemporal Nerve Syndrome (Frey’s Syndrome)
-Flushing with Pseudo carcinoid Syndrome in Secondary Male Hypogonadism
-Erysipelas
-Other Diseases Causing Episodic Flushing (including asthma, epilepsy and Lyme disease)
This girl has erythromelalgia and has burning, flushing and redness of the face:
Here a Mayo clinic doctor explains erythromelalgia. Although not everyone who went to the Mayo clinic with their EM symptoms were content with the medical help and 'trial and error' approach to treatment.
I recently read an interesting medical article, in which the author described a patient who had come to hospital with severe facial flushing and burning of the face and ears
"Erythema associated with pain and warmth on face and ears: a variant of erythermalgia or red ear syndrome?"
The article describes how Erythermalgia (another term for erythromelalgia) is a rare disorder of the skin, that is characterized by attacking of skin redness, pain and increased skin temperature. It mainly happens to the skin of the extremities, which means the hands, the feet and the face. It also can extend to the neck, face, ears and even the scrotum. The article describes the case of an 18-year-old woman who had a 3 years history of redness, pain and warmth of the skin of her face and ears only. Over time, the attacks (of flushing and heat and pain) became more regular and severe for her. She had these red hot flushing episodes on average several times a month, lasting from two to more than ten hours each time, and sometimes the attacks lasted days even. She noticed that the episodes always started on her cheeks and then radiated quickly to her ears and forehead. She did not get scales on her skin and she neither sweated or got a headache during these attacks. The most painful areas were the cheeks. The symptoms disappeared completely between episodes, and left no skin damage. When doctors examined her skin, they noticed skin redness (see picture from the patient on the left).
The researchers first considered the girl to have other health conditions that could cause the facial flushing and heat and redness, including seborrheic dermatitis (not the case as her skin showed no scales and flakes), relapsing polychondritis (inflammation of the nose and ear cartilage and other connective tissues in many organs, causing them to become inflamed and tender), chondrodermatitis nodularis chronica helicis, (another inflammatory condition affecting the ears). They ruled out contact dermatitis as well as photosensitivity, because the redness also calmed down again spontaneously, which is not usually seen with contact dermatitis and photosensitivity unless it is treated. The researchers especially suspected facial flushing to be a factor in this girls symptoms. But they stated that facial flushing is usually a symptom of an underlying medical condition or reaction to certain substance, like alcohol, drugs, allergy, emotions, exercise, food additives, skin disorders, etc.
"Patients with facial flushing experience a suddenly facial reddening, feel hot face, and always associate with other symptoms relating with the nosogenesis. The reddening may extend to the neck and upper chest, even the whole body, but the patients lack cutaneous pain. Harlequin syndrome is a benign condition showing a sudden onset of unilateral facial flushing and sweating, the present case lacked sweating and showed symmetrically facial redness associated with pain and warmth, Harlequin syndrome can also be excluded."
The doctors came finally to the conclusion that this patient had a rare form of erythermalgia (erythromelalgia). They also considered the option of Red Ear Syndrome (RES). Normally patients with this condition also suffer from red hot feet and hands. Not just a red hot face and ears. But in this case, doctors diagnosed her with a special form of erythromelalgia, that only affected her face and ears. They found out that the symptoms went down a bit (but not fully) after the patient was given oral antihistamine medication. The same happened when they applied topical steroid cream on her skin (be veeeery careful with this as a rosacea patient!! Steroid creams can worsen rosacea or even cause it in sensitive people with a predisposition for blushing and burning). Doctors also found that her skin improved mildly from a cream called Protopic (tacrolimus). However, what really helped control all these patients symptoms, was a combination treatment of aspirin and paroxetine; an SSRI type of antidepressant, also known under the names Paxil and Seroxat. Her face flushing and redness also improved markedly after having been treated with oral gabapentin (a nerve pain medication), and indomethacin (a non-steroid anti inflammatory drug), and topical lidocaine compounds (a local anesthetic (numbing medication) and cold masque for 3 days; but 2 weeks after she stopped using these three medications her symptoms returned. She was then treated with the aspirin 0.1, twice a day; paroxetine 10 mg daily, which resulted in complete disappearance of her symptoms after having been treated for 2 weeks. Without any treatment, the patient remained symptom free for 6 months and was still under follow-up. There are many treatment options for EM, but none work for everyone and they all have varying response success. No treatment is consistently effective, and to predict the efficacy for a specific treatment is also impossible. 
Lumbar sympathetic block was reported in a case showing successful response in a refractory EM recently. I will write another blog post soon about these sympathetic blocks for vascular rosacea. Most times, EM starts later in life, often in women's 50's or 60's. But interestingly enough, the case described, and another case referred to in the research above, mentions the onset of EM of only the face to be very young: 14 years of age and in the case of the girl described above 15 years of age. There has also been a case described by Ramirez and Kirsner of a man who's symptoms of ear burning started at the age of 50. The reasons for the younger age onset for EM of the face remains unknown, but because there are not that many cases of it known, it could also be a coincidence, the researchers wrote. They also think that dilation of the blood vessels in the skin is at least partially responsible for the symptoms this girl had. But unfortunately for us, they conclude that the exact mechanisms are not fully understood.
Erythromelalgia flushing of the face compared with my own rosacea flushing
Maybe a difference between these photos and the ones from myself is that I don't flush on my forehead and nose. I do get red flushed ears however, and red cheeks and chin. And lately my hands (fingers on both hands but especially on my right hand) glow red adn hot pretty much every evening.. I have to cool them in front of fan or in cold water. Need to see my derm again for re-check if my symptoms could be erythromelalgia (EM) instead of the Raynaud's Syndrome I was diagnosed with... My fingers don't go pale, only red and hot.
In the article above, researchers write that there have been more (rare) cases described in the medical literature of patients with erythromelalgia flushing of only the face and ears, and without the characteristic flushing and burning of hands and feet and legs.
"EM includes primary and secondary forms. It is considered that the mutations in SCN9A, encoding the sodium channel protein Na(v)1.7 subunit, are responsible for the primary type, while neuropathological and microvascular functional changes may be for the secondary one."
Red Ear Syndrome (RES) has some similar clinical features as EM. It always occurs in one, or less frequently in both ears. Sometimes the redness and flushing also radiates to the back of the neck, the forehead or even the jaw. Its attacks may be spontaneous, or triggered by touch, exertion, heat or cold stimuli, neck movements, stress, cleaning the ear, etc. RES also includes 2 subtypes: the primary and secondary. The primary form seems to start spontaneously and doctors have not found a cause for it yet. It commonly occurs in young people and is associated with migraine, while the secondary form happens more often to adults, and is linked to problems, injuries or pain of the neck disks. cervical disorders and pain or injuries of the jaw joint.
So erythromelalgia can look a lot like neurogenic (subtype 1) rosacea. Especially as in rare cases, erythromelalgia only manifests itself as redness and flushing and burning of the face (so not on the hands and feet as well). The case of the young woman described above showed the exact same symptoms as I have during a flare with (as it stands for now) the diagnosis rosacea.And some people with rosacea turn out to have erythromelalgia as well, for instance in the case of AEB:
AEB wrote on May 27th, 2016: "I started with facial flushing, which then spread to my ears, and a few months later chest and arms--brought on by stress but most of all heat. My diagnosis is rosacea and erythromelalgia because of the intensity of redness and burning and the times/ways it presents. So you may want to ask your doctor about that too and maybe do some research of your own (since it's rare and many drs haven't heard of it, I've found). The testing can be exhausting but gives you more treatment options and the relief of knowing what you're dealing with!"
More on Erythromelalgia (EM), a very rare cause of facial flushingErythromelalgia (EM) is a rare neurovascular disorder that typically affects the skin of the feet or hands, or both, and causes visible redness, intense heat and quite severe burning pain. It makes the blood vessels of the extremities (mostly hand and feet but also at times the face) episodically blocked, then become overly dilated and inflamed, causing throbbing heat, redness and burning pain. The pain is caused by the small fiber sensory nerves. The attacks are periodic and are commonly triggered by heat, pressure, mild activity, exertion, insomnia or stress. The term erythromelalgia describes the syndrome: erythros (redness), melos (extremity) and algia (pain). It can also affect the legs and arms or the face, nose and ears. Even in mild-to-moderate cases, normal functioning such as walking, standing, working, socializing, exercising, and sleeping may be impaired. Triggers for flare ups can be warm temperatures and even mild exercise. Cooling the hot body parts relieves the pain, as does elevating the affected areas. This is one of the hallmark characteristics of EM: cooling bringing relief.
The cause of EM is unknown in the vast majority of cases. Only 5% of patients is said to have a genetically inherited cause. Often the underlying engine and root problem for EM has to do with problems arising when nerves that carry messages to and from the brain and spinal cord from and to the rest of the body are damaged or diseased (peripheral neuropathies). And sometimes EM may be secondary to other disorders like the blood disease polycythemia. Recent research in the U.S. found the incidence of EM (the number of people a year diagnosed with EM) to be 1.3 per 100,000. The rate for women was higher – 2.0 per 100,000 per year – than men, which was just 0.6. The average age at diagnosis was 61 (source).
Like with rosacea, each case seems to react different to treatment options. Traditional over the counter pain medications or stronger prescription drugs help some. NSAID's and blood vessel constricting medication might help. Anticonvulsant drugs like Neurontin and Lyrica help others. Certain antidepressants like Cymbalta might help. According to the article above, aspirin 0.1, twice a day; paroxetine 10 mg daily help significantly for EM. Combinations of drugs also have been reported effective. For instance, Lyrica and Cymbalta, at the lowest possible dosage, have been reported to be more effective than either drug by itself. It is recommended that people with EM find a doctor willing to help them pursue a trial-and-error course of treatment
Raynaud's syndrome also gives red and flushed extremities (hands and feet typically). It is characterized by excessively reduced blood flow in response to cold or emotional stress, causing discoloration of the fingers, toes, and occasionally other areas. They can also swell a bit and start to throb or itch even. It can cause the fingers and feet to become pale, white and cold (usually when you are exposed to cold temperatures and the blood flow doesn't read the hands and feet), or for them to become purple (when oxygen supply is depleted) or red and hot (when the blood supply is up to normal again). Raynaud's and EM can go together sometimes. EM seems to give much more pain than Raynaud's and the discolorations and blood vessel constriction of toes and hands from Raynaud's tend to last a lot longer at a time, compared to the short EM attacks. However, some EM patients report attacks lasting from one hour to a few weeks even. Both can be triggered by cold and heat but EM can flare without any obvious trigger at play. People with EM complaint about random attacks, very red and swollen extremities which can feel like skin burns, yet also itch at times (probably due to the skin swelling and the nerves getting stimulated or trapped I can imagine). It prevents some patients even from working and sleeping and one patient details how she sometimes has to try to sleep with her hands raised in the air, in search of some relief (source). Read more on Raynaud's here.
Tests that you can ask your rheumatologist for
An ANA test - please ask him to specify that it be done by IFA methodology.
An ENA panel (includes SCL-70, and anti-RNP)
Anticentromere B test
Nailfold capillaroscopy (source).
EM lies within the field of 3 different medical specialisms, hematology, neurology and vascular diseases, which makes it even harder to treat, as very few specialist seems to master all for those 3 fields. You can read more on EM here. And here.
More pictures of what erythromelalgia
(EM) can look like
In this medical research paper, the case of a 33 year old woman is described with what doctors think is late-onset erythromelalgiaShe has flushing and burning of the face, ears, chest and sometimes hands. Her symptoms, as described below, sound very much like my own severe flushing and intensive lifestyle adaptations. "Erythromelalgia is a rare disorder characterized by episodic erythema and burning pain, which commonly involves the extremities. We present a case of late onset erythromelalgia in a previously healthy young woman and briefly review the literature. Our patient's case also has additional uncommon features not reported previously."
Case presentation
"A 33-year-old previously healthy Caucasian woman presented with complaints of episodic burning pain and flushing occurring in a central distribution involving her face, ears, upper chest and, occasionally, her upper extremities. Her symptoms were triggered by lying down or warm temperature exposure and were relieved by cooling measures. Extensive diagnostic work-up looking for secondary causes for the symptoms was negative and the diagnosis of erythromelalgia was made based on details provided in her clinical history supported by raised temperature in the affected area measured by thermography during a symptomatic episode. The patient did not respond to pharmacological therapy or surgical sympathectomy. She was advised on lifestyle modification to avoid activities which triggered her symptoms. She was hypothermic (she had very low body temperature) with a core temperature between 92 and 95°F. She also had premature ovarian failure, which had not previously been reported. She presented with photographs displaying bright erythema from her lower neck extending upwards to her face and head. Her symptoms were precipitated and worsened with lying down or warm temperature exposure, and were abated by cooling measures. She maintained her home air temperature at 60°F. She denied similar symptoms amongst family members, and there was no family history of EM. She was in good health until 2001 when she began to experience these painful burning and flushing episodes. Over time, the episodes increased in frequency and severity until presentation when she was experiencing multiple daily episodes, each lasting minutes to hours. Her symptoms were disabling and she was forced to leave her job. Physical examinations during multiple visits revealed normal vital signs except for a reduced core body temperature of 94° to 95°F. She had dry skin with a diffuse blanching erythema mainly over her face, and loss of her fingernails. She underwent an extensive evaluation which included a normal comprehensive metabolic panel and complete blood count (CBC) with differential. She did have a low free thyroxine level with a mildly elevated thyroid stimulating hormone level and was started on levothyroxine for primary hypothyroidism, but this did not change her symptoms. Approximately 1 year after the onset of symptoms, she developed oligomenorrhea and this later progressed to amenorrhea. She developed premature ovarian failure based on low estradiol levels and elevated luteinizing hormone (LH) and follicle-stimulating hormone (FSH). Ovarian ultrasound and pituitary magnetic resonance imaging (MRI) were normal. The autoimmune panel was normal including negative antiperoxidase, anti-adrenal, anti-ovarian, gastric parietal, antinuclear, anti-Smith, and anti-DNA antibodies.
Conclusion
"Erythromelalgia is a rare disorder of unknown cause. There is no confirmatory diagnostic test; diagnosis is based on details provided in the patient's medical history and physical examination during the episodes. For those affected, this disorder leads to significant long-term morbidity and unfortunately, to date, no definitive therapy is available except for lifestyle modification."
In the article, scientists also state that diagnosis of erythromelalgia is difficult and that there is no specific test for it. Doctors need to look at the symptoms and hear the history of the patient, to come to a diagnosis. They check for instance for increased skin temperature during a flushing attack. This is something also seen with rosacea flushing however. Many patients complain about their face feeling so hot, they reckon you can fry an egg on it. So there's some overlap of symptoms, therefore... The article continues that once a doctor suspects Erythromelalgia to be the cause of the patients symptoms, he or she needs to then exclude OTHER potential causes of flushing and burning of the face and extremities (hands and feet). Other causes associated with erythromelalgia include drugs such as verapamil, nicardipine, bromocriptine, pergolide, and mercury poisoning, and diseases such as systemic lupus erythematosus, Raynaud's disease, pernicious anemia, thrombotic thrombocytopenic purpura, infectious mononucleosis and diabetic neuropathy. They continue to write that the cause of EM is not fully known, but that research indicates that the cause has to do with not enough oxygen reaching the tissues because of a problem with the blood vessels not dilating and constricting as they should. Our body normally dictates our blood vessels how much they need to dilate or constrict in order to let enough blood go through the skin to keep our body temperature stable. And something goes wrong in this process with EM patients. EM patients have too much blood flow in fact in the smaller veins and capillaries of the extremities; the face, hands and feet. There is also a problem with sodium channels in the body staying open much more and longer than they normally should, adding to the neuropathic pain problem we see with EM. There is no known cure or all-round treatment. Doctors say to their EM patients that they should avoid their triggers and should cool their painful hot skin (safely, with fans and air conditioning and not with ice), including lifting up painful hot hands and feet, to stop the blood pooling. The authors of this article also mention that they know of several case reports where EM patients responded well to aspirin and intravenous (IV) administration of sodium nitroprusside and prostaglandins. Surgical sympathectomy (ETS for instance, read more about ETS and its risks in this blog post) has had variable results. Treatment with medications such as gabapentin, SSRIs, TCAs, and CCBs has had some symptomatic benefits in a few cases. One case study reported the efficacy of sodium channel blockers such as lidocaine and mexiletine, however in this specific article the authors state that their patient's symptoms did not improve with a trial of mexiletine therapy. She did have temporary improvement with misoprostol and clonidine, however beneficial effects were short-lived. She tries to control her symptom flares by keeping cool and setting her home temperature to 60°F. "Our patient remains unable to work and unable to tolerate any change in environment that exposes her to warm air." Well, that sounds like some of our rosaceans' lives :(
"Our patient's case has additional unusual features. Her pattern of involvement is mainly the upper chest, neck, face and head area, as opposed to the usual lower extremity involvement. She became hypothermic with a core temperature often in the 92-95°F range. This hypothermia is likely due to her chronic exposure and acclimation to a cool environment at home. An MRI scan of her brain was normal without any hypothalamic lesion, she had no other neurologic symptoms, and she hadn't suffered any head trauma. There was no prior history of body temperature dysregulation or poikilothermia. She had mild primary hypothyroidism with negative antithyroid antibodies. Lastly, she also developed premature ovarian failure, which has not been previously reported. We suggest that her low body temperature may be the cause of the premature ovarian failure."
I have been diagnosed back in 2009 with premature ovarian failure too, and reconfirmed a few years later. I always assumed the chronic inflammation in my body was to blame for it, but now I read that constantly keeping your body cold (I have done so since 1999 to avoid flushing and burning) is also being mentioned as a possible cause for this...
KRC wrote on May 4th 2014: "I am beginning to suspect that I might have something of this nature (EM) as in addition to horrific facial burning I appear to also have burn on my lower legs! The only confusing thing is I also get some of the minor breakouts associated w. rosacea. I am under a little bit of stress as life tends to do and am wondering if a plain old tranquilizer might help me - So many avenues to pursue. I wish the medical community were a little better educated to all of this. As it is with so many symptoms I feel like they just think I am nuts. And any mention of demodex makes them really squirm lol. It is classic as so many on the board have elaborated."
Meg wrote on May 4th 2014: "You are welcome. May I suggest you take a look the the EM site and if you want to discuss with others who have the same symptoms, join the following support group. I think you will find that many people with EM have both rosacea and Face EM, depending on the severity. If your legs are burning, along with your face, you probably have EM. It is primary EM is there is no other cause, but if you have something causing these symptoms, then it's seen as secondary. For instance EM can be caused by peripheral neuropathy, diabetes, lupus, etc,... EM is really a set of symptoms which are essentially red, hot, burning skin that is often relieved with cooling, etc,... More details on the first site listed above. Also, feel free to email me directly if I can be of further help."
GracieTiger wrote on December 19th 2008: "I need help understanding this - visit with derm NOT GOOD. Hi all - I just visited a dermatologist for the first time since I started flushing. He is recognized as a "top doc" in this city and came recommended on the rosacea site. For those of you who don't remember me, I'd like to give a brief history of my symptoms. I lived in rural Africa for a year, and about two weeks after returning, I started having a lot of unusual symptoms. the first of which was facial flushing. I had no history of flushing/blushing at all, ever. It started suddenly, and at first, it was pretty transient. More like blushing. Then it progressed to the point that I felt like my face was red and hot all the time. That feeling eventually subsided a bit, however, when that winter began, I realized that I had quite a problem on my hands. When my body was cold, then became warm, I would flush really really intensely for about eight to ten hours. the burning was near unbearable. without a doubt, every single time that I felt cold, then took a hot shower, or entered a hot room, I would have a major flush. It was pretty unbelievable to me that in just a couple months I went from never flushing to major major flushing.along with the flushing, I also had a lot of other problems. heart arrhythmia, panic attacks (no history at all), breathing problems, vision issues, joint swelling, etc etc. Eventually I was diagnosed with having a type of autonomic nervous system dysfunction - POTS. Over the summer, the flushing wasn't nearly as bad. My skin got kind of red in the summer, but I wouldn't say I flushed. no burning. Now that it is cold again, however, it is unbearable flushing. My body temp, during a flush, will go from like 96.5 pre-flush to 99 within ten minutes of flushing. I don't know if this is rosacea or not. But it doesn't really matter what it is, it is debilitating. I used to be a zookeeper and was working really really hard to be a wildlife veterinarian. that dream is shot out the door due to flushing. I can no longer work outside. I used to be an avid winter sportswoman, now I cannot do any outdoor activities. I used to love mountain camping, but now I can't do that, ever, because it gets too cold at night to camp and I flush when it warms up in the day. So, I finally went and saw a dermatologist. after educating myself about rosacea, flushing, blood vessel damage, etc. I felt like I would be a good candidate for some laser treatment. my dermatologist completely write me off.
He kept saying that it's clear I don't have rosacea because I have no inflammation and because I was not red at the time I saw him. He said it is clearly an autonomic nervous system dysfunction, and that he had no idea why I thought he could help me.
He completely belittled me, repeatedly asking what I thought i would get out of him. when I tried to share my knowledge of blood vessel damage from prolonged flushing and the benefits of laser in curbing that damage, he told me I sound too educated to think he could do anything for me. Again, this is the top-rated dermatologist in my area. that is fine if he feels there is nothing that can be done for me. I was not expecting a miracle cure. but to repeatedly ask me why I came to see him, that was crossing the asshole line. I tried to explain how much I have lost in my life due to this flushing and he kept telling me that my other issues (heart problems, etc) are the ones I should be concerned about. Yes, I agree, and I do worry about those too, but the psychological impact that the flushing has had on my life is far greater than the other issues. What killed me the most was when my doctor said he would never want rosacea so I should be glad I don't have it. Whatever the name of what I have is, flushing is flushing!! Plus, if he would never want rosacea, then how dare he question me as to why I am visiting him for assistance. Obviously the flushing is pretty darn damaging to me too. I don't know how much longer I can go on with this flushing with no treatment at all. The propranolol helps a little, but not much. I can't even blow dry my hair and straighten it anymore. I look like a crazed mountain woman. Haha, not really, but I would love to be able to straighten my hair once in a while without knowing that I will be red and burning for ten hours. So I am really venting my frustrations, because I am really really frustrated right now, but also hoping you all can help me. Given that I flush, and have been intensely flushing for about two years for hours at a time, am I really not a candidate for laser? Even if I don't have rosacea? Let's say that the autonomic dysfunction is causing all my problems, can't flushing alone be reason for laser? Wouldn't blocking the newly formed or damaged blood vessels help stop the burning, at least? The derm also pointed out that flushing can become rosacea eventually. So don't I want to treat it early to prevent it from crossing that point? And how does he know it hasn't already? Just because I don't have bumps? I didn't think that was a criteria for rosacea. don't many of you just have flushing? And isn't it important to treat it early? Wouldn't i want to start now in order to prevent it from ever becoming rosacea? Soooo frustrated. my doc's solution: move to southern California, Hawaii or Florida. great, huh? Oh sure, let me just pack up my bags now!!! Thanks for listening."
Meg replied: "Sounds a lot like you do in fact have an autonomic nervous system dysfunction. I have severe and painful flushing that was diagnosed at the Mayo Clinic as a form of autonomic dysfunction. It turns out that I also stopped sweating, which is another autonomic problem. You might consider seeing the autonomic neurologists at Mayo for an accurate diagnosis. Also, I find my flushing to be more like erythromelalgia, rather than rosacea, due to the burning pain. If you have red hot burning pain when you flush, look at http://www.erythromelalgia.org/. Hope this helps. Best regards, Meg"
And also: "Erythromelalgia (EM) definitely includes the face. If you look at the EM (TEA) website, you will see they even post photos of people who have EM on their faces. Having a face only case is rare. Most people seem to have the face involved in addition to hands and feet, but some cases also involve the face or even the torso. Any part can be affected. EM is noted or defined as red, hot burning skin that is relieved by cooling. Generally, it is a form of autonomic nervous system dysfunction. GracieTiger, my flushing/EM is face only. I have no symptoms on my hands, feet or other body parts. Just my face and ears. Hope this clears things up. Meg. PS - I have cut and pasted the info below from the EM (TEA) website (note the inclusion of the face under the "Location" paragraph - Symptoms; If you have been diagnosed with EM, symptoms may include hands or feet that are very red to purple in color, are perhaps swollen, hot to the touch, and have burning pain. Location; For some, EM symptoms may appear in the face, ears, knees or other parts of the body. The intensity of the symptoms varies from person to person. Some notice a continual burning pain while others are troubled with "flare-ups" or episodes lasting from minutes to days in length. Triggers; Warm temperatures seem to be the most frequent trigger for EM episodes. Flare-ups are provoked by heat and exercise, and symptoms are relieved by cooling and elevating the affected extremities. Some TEA members have found that foods, spices like MSG, beverages (particularly alcohol) and some drugs can make EM symptoms worse."
GinaK wrote on March 2015: "I have been battling with my condition for about 7 years. I had a battery of tests for allergies, and eventually my GP diagnosed (at my suggestion) that I had a nasty case of rosacea. Now however, I'm not so sure. I am suffering an extreme flare up - I arrived in Australia, Gold Coast, to visit my son, a week ago, from the UK. It was unseasonably hot. Within 3-4 days I knew for sure I was going to have a flare. And, sadly, this has been the worst one I've had for years - in fact the most similar was the first time I had it. There is a very distinct pattern to a flare..so if you can bear with me I'll describe it, as just maybe it might help someone to identify it as one thing over another - ie is it rosacea or erythromelalgia. I can 'feel' it coming..I get a distinctive tingling in my flesh, particularly in my lips and in my 'nose to mouth' creases. Then my face starts to heat up, and it gets really properly burningly hot, and of course it goes very very very red. It hurts. Then it swells up (in the attached pictures the swelling has actually gone down a bit). That hurts too. This time the swelling went right up into my eye sockets. I looked like I'd been in a punch up. The pain is an intense burning sensation, there's some itchiness, and there's a sort of deep ache..that's not quite right..it's so tender and ultra sensitive, even to just moving around normally hurts in this odd aching way. I manage it by trying to cool it with damp cloths, splash cool water on, sometimes I freeze a bottle with water and use that as a cooling pack - this brings real relief (I am quite careful to not go overboard with very icy cooling as I know that can be damaging, but it's hard sometimes as I simply feel like my face is on fire). As it happens, my son lives with a doctor, he recommended to use ibuprofen and/or aspirin to ease the 'inflammation' and to take anti-histamine as well. I'm taking these as I can't see what harm they can do but it's impossible to know if they make any significant difference. And then keep cool, out of the sun until things get better. Once this passes, I will be using 50+ sunblock (not sunscreen), and I have a large hat. Just to be clear, I didn't spend any amount of time in the hot sun when I arrived and did use 50+ sunscreen as well. This time I didn't get a symptom that I usually do, which is hot, burning swelling ears. But I did have a strange one that I haven't had for a long time, which is a very odd tingling itchiness in my lower forearms and legs. The other thing I do is to use some kind of cream to ease my poor ruined skin. Once the flare has peaked, and is beginning to cool off and the swelling eases, the top layer of skin starts to peel and flake. It's very unpleasant...it is very hard and dry and uncomfortable if I don't keep it 'lubricated' as it were. Most things are too painful to put on my skin at this stage, but oddly vaseline with cocoa butter has been a boon this time. Absolutely no stinging and it has helped me manage the 'shedding' stage much more easily. That's why my face is so shiny in the picture. I stumbled across something about erythromelalgia in a rosacea forum, and the symptoms - especially the burning of my ears - seem to fit a bit better. I will definitely be running this past my GP when I get home. But...if anyone has any advice or help I'd be so very grateful. I try not to let it get me down, but it has a massive impact on my life - there are a few days where I don't really go out, while it sorts itself out. I have had to miss work - I like to think I'm not vain, but I do feel very self conscious about it and can't help wondering what people must think (ie I'm a drunk..I don't drink alcohol at all because guess what...it makes me flush bright red!..). Anyway. Big sigh. I look forward to hearing from the community. You all have my sympathy and best wishes with your conditions xxxx"
Nel replied on March 2015: "GinaK, I am so sorry. That looks terribly painful. I have no experience of rosacea so can’t say if this looks more like EM. I have facial flares but not that severe or extensive, but I have heard a description of facial EM which was much like yours. Which is not to say that she can’t have been wrongly diagnosed with EM. It was brave of you to travel to the Gold Coast and bad luck that they have had such heat lately. I found the Gold Coast and points further north too hot for me even before I developed EM and I haven’t been out in sunshine longer than it takes to get in the car on my driveway for many years. I hope members can give your some answers."
Sheltielife replied on March 2015: "My face looks like that sometimes I was always being asked in the winter where had I been on holiday. People obviously thought it was sun burn as that’s what it looks like and in a way the pain is similar to really bad sun burn."
Dragonfly replied on March 2015: "Hi Gina, I have had EM for about 18 months. A few weeks ago I started to get facial flares when I got hot. I do not have any other rosacea features like the typical facial redness over the nose and cheeks. I drink lots of water and try to keep my body temp down. (This all happened in our cold winter when I went outside and was likely overdressed) I use cool packs on my face when it flares. I was reading about a new type of rosacea called neurogenic rosacea. This new category sounds to me like facial EM. My rheumatologist is sending me to a dermatologist to confirm the diagnosis of facial EM. I already have a diagnosis of EM in my feet. I also have small fibre neuropathy confirmed on skin biopsy. I am so sorry that you having this trouble. Hopefully you can get things sorted out soon. Cheers Dragonfly"
Viking replied on March 2015: "My EM involved only my feet and hands until I began having terrible flares involving my face and ears during menopause. Avoiding triggers (coffee, red wine, heat, steamed up bathroom, changing weather etc.) is obviously very important but there was and still is a complete randomness to some flares. Once a flare is to the intense state, I've never found anything to stop it but just wait it out with fans and refrigerated gel packs. Five years later it is better - I still have flares but the extremely intense ones happen less often. I don't know why it's improved so can't attribute it to any medication or treatment. I am grateful that it seems to be better but I am chronically apprehensive and spooked by the slightest tingle in my ear lobe or any warm sensation on my face. I am so sorry to read of your condition, GinaK, and hope that you will find relief."
Alina_Delp replied on March 2015: "I’m so sorry. That does look terrible! I can’t say for sure because I don’t have experience with rosacea. EM is often misdiagnosed as rosacea or other skin sensitivity at first as was mine. This is definitely worth taking EM info to your doctor and discussing this with them. I how one way or another you are able to find help soon."
Lynne replied on March 2015: "Dear GinaK, Your face reminds me of my own, most unfortunately, when I have an especially bad episode. Plus your description of feeling like your face is on fire -- exactly the words I use. In some ways, the burning ears are even worse than the face. Like you, I've gotten diagnosed with rosacea, but the condition has never responded to typical rosacea treatments. Correction -- not to most. Recently I've tried a product for general redness as well as rosacea, and it seems to have helped (I'll go look up the name before I close this note). But because I run "hot" all the time, I usually use it just overnight. In addition, the top dermal layer (one of my dr. calls it the dermal matrix) was apparently damaged by a couple of events over the past decade and a half: first, I mowed through poison ivy unwittingly and ended up with a systemic infection, along with extreme skin involvement ... I blistered badly on my face and forearms. Second, I tried a DIY facial peel product for brown spots ... my face blew up even worse than your in the photos, and now when I "flare," the pattern follows the destruction from these two events. And THEN, I got bitten by a tick and developed a raging Lyme infection. The bite was on my midriff, and the rashing was worst on my upper body and face. I successfully killed off the Lyme, but then had almost a year of trying to resolve the Candida infection that was toe-to-forehead on my skin (nothing internal, strangely enough). That rash again followed the lines of the other events ... which is when my dr. declared the dermal matrix was irreparably damaged ... any rashing was following the lines of least resistance, whether rosacea or erythromelalgia. So maybe I have both conditions (the rosacea was first diagnosed years and years previous to the poison ivy infection, based only on a red nose and one little bump that wouldn't heal; retin A prescribed but it was too drying). But the severe burning seems more like the EM to me ... I've done a LOT of research. Like you, I have what's called the "prodrome" -- the early warning symptoms, and I know what's coming. When I researched EM, I found that some dr. believe it's related to nerves in the mid-back -- though this may be more about excess sweating than EM (even as a kid I've broken a sweat faster than anyone else around me, and it tends to be very heavy on my face -- combine this with the flushing and I often have people afraid I'm having a heart attack!). Anyway, there is surgery that can be done, but it's one of those 50/50 things: 50 % chance it helps, 50% chance it makes things worse ... and it's not uncommon for repeat surgery to be needed/done. To me, it's not worth the risk, although it's a close call at times! As you do, when I am especially bothered by the heat and swelling, I use cold packs, carefully. I sometimes get relief from aspirin and/or Benadryl. Recently, I've been developing a rash on lower arms (again, location where poison ivy was especially bad) and upper chest. So far, these areas don't get hot or burn, but I wonder if it's just a matter of time before they do. I've gotten so I avoid going places where I'd expect to be particularly hot and/or humid because either of those conditions will set me off 100% badly. As it is, sometimes in air conditioned stores, and in my a/c home, for seemingly no reason, I flare. Some incidences are pretty short-lived, and I seem to cycle into periods when every one of them is severe and lasts for hours. None of this is helpful, I'm afraid, but I wanted to assure you that there are people who know exactly what you go through. When I am regular about taking them, several supplements usually help (both reduce how often I flare, and the severity of flares)." hesperidin and ginger (which is both an anti-inflammatory and a vaso-constrictor). There is another that slips my mind at the moment. Oh, the cream I spoke of earlier is "Facial Redness Repair," by TriDerma. I bought it online when I couldn't get more of another product by RosaceaCare (as I recall) that contains Strontium. [..] I found several articles that link gluten/celiac problem with EM ... and something called small nerve neuropathy. Often someone with EM can be tested and found to have this type of neuropathy which is known to involve the face (unlike the more common peripheral neuropathy of the feet, secondarily, of the hands). And on your note about menopause -- my hot flashes/flushes really amped up as I approached it at age 55, but I had night sweats starting at 39. I am now 60, and I still believe I have hormonal swings that bring on a different kind of flushing vs. the EM ... this despite using bio-identical hormone replacement (but can't tolerate traditional estrogen). Good luck to you ... hope whatever it is you've got doesn't get any worse. :-)"
On a website called Melissaannsite, the author describes four (young) cases of erythromelalgia
She writes for instance: "Imagine being told by a doctor that your child has a horrific syndrome – one that you have never heard of and one that will leave your child feeling like they have been set on fire and then left with second-degree burns. Where do you, the parent, begin? Can you even try to envision this intensity of pain? While not all rare diseases are fatal, the suffering that accompanies a condition called Erythromelalgia (EM) is life changing and can cause severe impairment. Many sufferers are left disabled, some commit suicide as a result. EM causes severe burning pain, visible redness (erythema) of the skin, swelling, and increased skin temperature, particularly of the hands and feet. However, the face, ears, and limbs can also be affected. Some people burn continuously, whilst others have intermittent episodes of “flaring” throughout the day. The excruciating flares can last from hours to days at a time. The disease does not discriminate by age, ethnicity or gender. The exact underlying cause of EM remains unknown. However, one theory maintains the condition results from vasomotor abnormalities. These abnormalities affect the normal narrowing (constriction) and widening (dilation) of the diameter (caliber) of certain blood vessels, which leads to impaired blood flow to the extremities or other body parts." (Scarlet Nat: This sounds surprisingly similar to what seems to be causing neurogenic rosacea flushing and burning, as far as we know now, although rosacea - like EM - has many possible triggers that can fire off those blood vessels to dilate). She describes the lives of five young EM patients.
GracieTiger wrote on December 19th 2008: "I need help understanding this - visit with derm NOT GOOD. Hi all - I just visited a dermatologist for the first time since I started flushing. He is recognized as a "top doc" in this city and came recommended on the rosacea site. For those of you who don't remember me, I'd like to give a brief history of my symptoms. I lived in rural Africa for a year, and about two weeks after returning, I started having a lot of unusual symptoms. the first of which was facial flushing. I had no history of flushing/blushing at all, ever. It started suddenly, and at first, it was pretty transient. More like blushing. Then it progressed to the point that I felt like my face was red and hot all the time. That feeling eventually subsided a bit, however, when that winter began, I realized that I had quite a problem on my hands. When my body was cold, then became warm, I would flush really really intensely for about eight to ten hours. the burning was near unbearable. without a doubt, every single time that I felt cold, then took a hot shower, or entered a hot room, I would have a major flush. It was pretty unbelievable to me that in just a couple months I went from never flushing to major major flushing.along with the flushing, I also had a lot of other problems. heart arrhythmia, panic attacks (no history at all), breathing problems, vision issues, joint swelling, etc etc. Eventually I was diagnosed with having a type of autonomic nervous system dysfunction - POTS. Over the summer, the flushing wasn't nearly as bad. My skin got kind of red in the summer, but I wouldn't say I flushed. no burning. Now that it is cold again, however, it is unbearable flushing. My body temp, during a flush, will go from like 96.5 pre-flush to 99 within ten minutes of flushing. I don't know if this is rosacea or not. But it doesn't really matter what it is, it is debilitating. I used to be a zookeeper and was working really really hard to be a wildlife veterinarian. that dream is shot out the door due to flushing. I can no longer work outside. I used to be an avid winter sportswoman, now I cannot do any outdoor activities. I used to love mountain camping, but now I can't do that, ever, because it gets too cold at night to camp and I flush when it warms up in the day. So, I finally went and saw a dermatologist. after educating myself about rosacea, flushing, blood vessel damage, etc. I felt like I would be a good candidate for some laser treatment. my dermatologist completely write me off.
He kept saying that it's clear I don't have rosacea because I have no inflammation and because I was not red at the time I saw him. He said it is clearly an autonomic nervous system dysfunction, and that he had no idea why I thought he could help me. He completely belittled me, repeatedly asking what I thought i would get out of him. when I tried to share my knowledge of blood vessel damage from prolonged flushing and the benefits of laser in curbing that damage, he told me I sound too educated to think he could do anything for me. Again, this is the top-rated dermatologist in my area. that is fine if he feels there is nothing that can be done for me. I was not expecting a miracle cure. but to repeatedly ask me why I came to see him, that was crossing the asshole line. I tried to explain how much I have lost in my life due to this flushing and he kept telling me that my other issues (heart problems, etc) are the ones I should be concerned about. Yes, I agree, and I do worry about those too, but the psychological impact that the flushing has had on my life is far greater than the other issues. What killed me the most was when my doctor said he would never want rosacea so I should be glad I don't have it. Whatever the name of what I have is, flushing is flushing!! Plus, if he would never want rosacea, then how dare he question me as to why I am visiting him for assistance. Obviously the flushing is pretty darn damaging to me too. I don't know how much longer I can go on with this flushing with no treatment at all. The propranolol helps a little, but not much. I can't even blow dry my hair and straighten it anymore. I look like a crazed mountain woman. Haha, not really, but I would love to be able to straighten my hair once in a while without knowing that I will be red and burning for ten hours. So I am really venting my frustrations, because I am really really frustrated right now, but also hoping you all can help me. Given that I flush, and have been intensely flushing for about two years for hours at a time, am I really not a candidate for laser? Even if I don't have rosacea? Let's say that the autonomic dysfunction is causing all my problems, can't flushing alone be reason for laser? Wouldn't blocking the newly formed or damaged blood vessels help stop the burning, at least? The derm also pointed out that flushing can become rosacea eventually. So don't I want to treat it early to prevent it from crossing that point? And how does he know it hasn't already? Just because I don't have bumps? I didn't think that was a criteria for rosacea. don't many of you just have flushing? And isn't it important to treat it early? Wouldn't i want to start now in order to prevent it from ever becoming rosacea? Soooo frustrated. my doc's solution: move to southern California, Hawaii or Florida. great, huh? Oh sure, let me just pack up my bags now!!! Thanks for listening."
Meg replied: "Sounds a lot like you do in fact have an autonomic nervous system dysfunction. I have severe and painful flushing that was diagnosed at the Mayo Clinic as a form of autonomic dysfunction. It turns out that I also stopped sweating, which is another autonomic problem. You might consider seeing the autonomic neurologists at Mayo for an accurate diagnosis. Also, I find my flushing to be more like erythromelalgia, rather than rosacea, due to the burning pain. If you have red hot burning pain when you flush, look at http://www.erythromelalgia.org/. Hope this helps. Best regards, Meg"And also: "Erythromelalgia (EM) definitely includes the face. If you look at the EM (TEA) website, you will see they even post photos of people who have EM on their faces. Having a face only case is rare. Most people seem to have the face involved in addition to hands and feet, but some cases also involve the face or even the torso. Any part can be affected. EM is noted or defined as red, hot burning skin that is relieved by cooling. Generally, it is a form of autonomic nervous system dysfunction. GracieTiger, my flushing/EM is face only. I have no symptoms on my hands, feet or other body parts. Just my face and ears. Hope this clears things up. Meg. PS - I have cut and pasted the info below from the EM (TEA) website (note the inclusion of the face under the "Location" paragraph - Symptoms; If you have been diagnosed with EM, symptoms may include hands or feet that are very red to purple in color, are perhaps swollen, hot to the touch, and have burning pain. Location; For some, EM symptoms may appear in the face, ears, knees or other parts of the body. The intensity of the symptoms varies from person to person. Some notice a continual burning pain while others are troubled with "flare-ups" or episodes lasting from minutes to days in length. Triggers; Warm temperatures seem to be the most frequent trigger for EM episodes. Flare-ups are provoked by heat and exercise, and symptoms are relieved by cooling and elevating the affected extremities. Some TEA members have found that foods, spices like MSG, beverages (particularly alcohol) and some drugs can make EM symptoms worse."
GinaK wrote on March 2015: "I have been battling with my condition for about 7 years. I had a battery of tests for allergies, and eventually my GP diagnosed (at my suggestion) that I had a nasty case of rosacea. Now however, I'm not so sure. I am suffering an extreme flare up - I arrived in Australia, Gold Coast, to visit my son, a week ago, from the UK. It was unseasonably hot. Within 3-4 days I knew for sure I was going to have a flare. And, sadly, this has been the worst one I've had for years - in fact the most similar was the first time I had it. There is a very distinct pattern to a flare..so if you can bear with me I'll describe it, as just maybe it might help someone to identify it as one thing over another - ie is it rosacea or erythromelalgia. I can 'feel' it coming..I get a distinctive tingling in my flesh, particularly in my lips and in my 'nose to mouth' creases. Then my face starts to heat up, and it gets really properly burningly hot, and of course it goes very very very red. It hurts. Then it swells up (in the attached pictures the swelling has actually gone down a bit). That hurts too. This time the swelling went right up into my eye sockets. I looked like I'd been in a punch up. The pain is an intense burning sensation, there's some itchiness, and there's a sort of deep ache..that's not quite right..it's so tender and ultra sensitive, even to just moving around normally hurts in this odd aching way. I manage it by trying to cool it with damp cloths, splash cool water on, sometimes I freeze a bottle with water and use that as a cooling pack - this brings real relief (I am quite careful to not go overboard with very icy cooling as I know that can be damaging, but it's hard sometimes as I simply feel like my face is on fire). As it happens, my son lives with a doctor, he recommended to use ibuprofen and/or aspirin to ease the 'inflammation' and to take anti-histamine as well. I'm taking these as I can't see what harm they can do but it's impossible to know if they make any significant difference. And then keep cool, out of the sun until things get better. Once this passes, I will be using 50+ sunblock (not sunscreen), and I have a large hat. Just to be clear, I didn't spend any amount of time in the hot sun when I arrived and did use 50+ sunscreen as well. This time I didn't get a symptom that I usually do, which is hot, burning swelling ears. But I did have a strange one that I haven't had for a long time, which is a very odd tingling itchiness in my lower forearms and legs. The other thing I do is to use some kind of cream to ease my poor ruined skin. Once the flare has peaked, and is beginning to cool off and the swelling eases, the top layer of skin starts to peel and flake. It's very unpleasant...it is very hard and dry and uncomfortable if I don't keep it 'lubricated' as it were. Most things are too painful to put on my skin at this stage, but oddly vaseline with cocoa butter has been a boon this time. Absolutely no stinging and it has helped me manage the 'shedding' stage much more easily. That's why my face is so shiny in the picture. I stumbled across something about erythromelalgia in a rosacea forum, and the symptoms - especially the burning of my ears - seem to fit a bit better. I will definitely be running this past my GP when I get home. But...if anyone has any advice or help I'd be so very grateful. I try not to let it get me down, but it has a massive impact on my life - there are a few days where I don't really go out, while it sorts itself out. I have had to miss work - I like to think I'm not vain, but I do feel very self conscious about it and can't help wondering what people must think (ie I'm a drunk..I don't drink alcohol at all because guess what...it makes me flush bright red!..). Anyway. Big sigh. I look forward to hearing from the community. You all have my sympathy and best wishes with your conditions xxxx"Nel replied on March 2015: "GinaK, I am so sorry. That looks terribly painful. I have no experience of rosacea so can’t say if this looks more like EM. I have facial flares but not that severe or extensive, but I have heard a description of facial EM which was much like yours. Which is not to say that she can’t have been wrongly diagnosed with EM. It was brave of you to travel to the Gold Coast and bad luck that they have had such heat lately. I found the Gold Coast and points further north too hot for me even before I developed EM and I haven’t been out in sunshine longer than it takes to get in the car on my driveway for many years. I hope members can give your some answers."
Sheltielife replied on March 2015: "My face looks like that sometimes I was always being asked in the winter where had I been on holiday. People obviously thought it was sun burn as that’s what it looks like and in a way the pain is similar to really bad sun burn."Dragonfly replied on March 2015: "Hi Gina, I have had EM for about 18 months. A few weeks ago I started to get facial flares when I got hot. I do not have any other rosacea features like the typical facial redness over the nose and cheeks. I drink lots of water and try to keep my body temp down. (This all happened in our cold winter when I went outside and was likely overdressed) I use cool packs on my face when it flares. I was reading about a new type of rosacea called neurogenic rosacea. This new category sounds to me like facial EM. My rheumatologist is sending me to a dermatologist to confirm the diagnosis of facial EM. I already have a diagnosis of EM in my feet. I also have small fibre neuropathy confirmed on skin biopsy. I am so sorry that you having this trouble. Hopefully you can get things sorted out soon. Cheers Dragonfly"
Viking replied on March 2015: "My EM involved only my feet and hands until I began having terrible flares involving my face and ears during menopause. Avoiding triggers (coffee, red wine, heat, steamed up bathroom, changing weather etc.) is obviously very important but there was and still is a complete randomness to some flares. Once a flare is to the intense state, I've never found anything to stop it but just wait it out with fans and refrigerated gel packs. Five years later it is better - I still have flares but the extremely intense ones happen less often. I don't know why it's improved so can't attribute it to any medication or treatment. I am grateful that it seems to be better but I am chronically apprehensive and spooked by the slightest tingle in my ear lobe or any warm sensation on my face. I am so sorry to read of your condition, GinaK, and hope that you will find relief."
Alina_Delp replied on March 2015: "I’m so sorry. That does look terrible! I can’t say for sure because I don’t have experience with rosacea. EM is often misdiagnosed as rosacea or other skin sensitivity at first as was mine. This is definitely worth taking EM info to your doctor and discussing this with them. I how one way or another you are able to find help soon."
Lynne replied on March 2015: "Dear GinaK, Your face reminds me of my own, most unfortunately, when I have an especially bad episode. Plus your description of feeling like your face is on fire -- exactly the words I use. In some ways, the burning ears are even worse than the face. Like you, I've gotten diagnosed with rosacea, but the condition has never responded to typical rosacea treatments. Correction -- not to most. Recently I've tried a product for general redness as well as rosacea, and it seems to have helped (I'll go look up the name before I close this note). But because I run "hot" all the time, I usually use it just overnight. In addition, the top dermal layer (one of my dr. calls it the dermal matrix) was apparently damaged by a couple of events over the past decade and a half: first, I mowed through poison ivy unwittingly and ended up with a systemic infection, along with extreme skin involvement ... I blistered badly on my face and forearms. Second, I tried a DIY facial peel product for brown spots ... my face blew up even worse than your in the photos, and now when I "flare," the pattern follows the destruction from these two events. And THEN, I got bitten by a tick and developed a raging Lyme infection. The bite was on my midriff, and the rashing was worst on my upper body and face. I successfully killed off the Lyme, but then had almost a year of trying to resolve the Candida infection that was toe-to-forehead on my skin (nothing internal, strangely enough). That rash again followed the lines of the other events ... which is when my dr. declared the dermal matrix was irreparably damaged ... any rashing was following the lines of least resistance, whether rosacea or erythromelalgia. So maybe I have both conditions (the rosacea was first diagnosed years and years previous to the poison ivy infection, based only on a red nose and one little bump that wouldn't heal; retin A prescribed but it was too drying). But the severe burning seems more like the EM to me ... I've done a LOT of research. Like you, I have what's called the "prodrome" -- the early warning symptoms, and I know what's coming. When I researched EM, I found that some dr. believe it's related to nerves in the mid-back -- though this may be more about excess sweating than EM (even as a kid I've broken a sweat faster than anyone else around me, and it tends to be very heavy on my face -- combine this with the flushing and I often have people afraid I'm having a heart attack!). Anyway, there is surgery that can be done, but it's one of those 50/50 things: 50 % chance it helps, 50% chance it makes things worse ... and it's not uncommon for repeat surgery to be needed/done. To me, it's not worth the risk, although it's a close call at times! As you do, when I am especially bothered by the heat and swelling, I use cold packs, carefully. I sometimes get relief from aspirin and/or Benadryl. Recently, I've been developing a rash on lower arms (again, location where poison ivy was especially bad) and upper chest. So far, these areas don't get hot or burn, but I wonder if it's just a matter of time before they do. I've gotten so I avoid going places where I'd expect to be particularly hot and/or humid because either of those conditions will set me off 100% badly. As it is, sometimes in air conditioned stores, and in my a/c home, for seemingly no reason, I flare. Some incidences are pretty short-lived, and I seem to cycle into periods when every one of them is severe and lasts for hours. None of this is helpful, I'm afraid, but I wanted to assure you that there are people who know exactly what you go through. When I am regular about taking them, several supplements usually help (both reduce how often I flare, and the severity of flares)." hesperidin and ginger (which is both an anti-inflammatory and a vaso-constrictor). There is another that slips my mind at the moment. Oh, the cream I spoke of earlier is "Facial Redness Repair," by TriDerma. I bought it online when I couldn't get more of another product by RosaceaCare (as I recall) that contains Strontium. [..] I found several articles that link gluten/celiac problem with EM ... and something called small nerve neuropathy. Often someone with EM can be tested and found to have this type of neuropathy which is known to involve the face (unlike the more common peripheral neuropathy of the feet, secondarily, of the hands). And on your note about menopause -- my hot flashes/flushes really amped up as I approached it at age 55, but I had night sweats starting at 39. I am now 60, and I still believe I have hormonal swings that bring on a different kind of flushing vs. the EM ... this despite using bio-identical hormone replacement (but can't tolerate traditional estrogen). Good luck to you ... hope whatever it is you've got doesn't get any worse. :-)"
She writes for instance: "Imagine being told by a doctor that your child has a horrific syndrome – one that you have never heard of and one that will leave your child feeling like they have been set on fire and then left with second-degree burns. Where do you, the parent, begin? Can you even try to envision this intensity of pain? While not all rare diseases are fatal, the suffering that accompanies a condition called Erythromelalgia (EM) is life changing and can cause severe impairment. Many sufferers are left disabled, some commit suicide as a result. EM causes severe burning pain, visible redness (erythema) of the skin, swelling, and increased skin temperature, particularly of the hands and feet. However, the face, ears, and limbs can also be affected. Some people burn continuously, whilst others have intermittent episodes of “flaring” throughout the day. The excruciating flares can last from hours to days at a time. The disease does not discriminate by age, ethnicity or gender. The exact underlying cause of EM remains unknown. However, one theory maintains the condition results from vasomotor abnormalities. These abnormalities affect the normal narrowing (constriction) and widening (dilation) of the diameter (caliber) of certain blood vessels, which leads to impaired blood flow to the extremities or other body parts." (Scarlet Nat: This sounds surprisingly similar to what seems to be causing neurogenic rosacea flushing and burning, as far as we know now, although rosacea - like EM - has many possible triggers that can fire off those blood vessels to dilate). She describes the lives of five young EM patients.
Sebastian has the inherited form of EM, meaning he was born with a mutation in the sodium channel gene (SCN9A). He has the genetic form of erythromelalgia together with small fiber polyneuropathy. It was in 2009 that Sebastian experienced what seemed to be a jolt of neurological pain in his leg. Although the pain only lasted a few seconds, it caused him to fall to the ground screaming. He suffered from episodes like this intermittently over the next five years. In January 2015, Sebastian started experiencing severe burning in his hands and feet along with shooting pains, which limited his ability to walk. His hands and feet were bright red and burned 24 hours a day. Even the touch of clothing on his skin would trigger pain in his body. He typically left school halfway through the day crying in pain. Eventually, he ended up confined to a chair with a bucket of cold water and fans blowing on him all day. Sleeping was nearly impossible. Eventually he was put on a lidocaine infusion to help stabilize his pain. It is quite difficult to treat a child with EM because strong medications are rarely, if ever, administered to pediatrics. He was slowly transitioned to oral mexiletine. Mexiletine is a non-selective voltage-gated sodium channel blocker, which belongs to the Class IB antiarrhythmic group of medicines. It is used to treat arrhythmias within the heart or seriously irregular heartbeats. This is a rarely given off-label treatment. Sebastian felt incredibly isolated and alone. Play dates did not exist anymore and he lost friendships. He was a prisoner in his own house. His mother felt helpless so she decided to do something about it. She opened a post office box. Then she wrote about her son’s situation on social media and asked for letters or cards with words of encouragement. Before she knew it, Sebastian was receiving cards, drawings, toys, letters and care packages from all over the world. It put a smile on his face over the next few months. About six months later, in early 2016, Sebastian finally had a treatment dosage that helped the burning subside. He was able to return to a somewhat normal life but with limitations, of course. He must take his medications (mexiletine and gabapentin) every four hours around the clock. Although his parents are worried about the long-term effects of the medication, they are more concerned with giving him a good quality of life. He still experiences daily pain daily but not nearly as bad as in the years prior to treatment. He is most affected now by heat and fluctuations in temperatures and experiences worse flares in the winter (as do many EM sufferers).
James Dennee, 7 years old, was diagnosed with EM in 2015. During his first year with EM, he missed so many days of school due to pain that his mother, Shanna, who also has EM, decided to devote her time to homeschooling her son. Vacations are no longer enjoyable because James fears the burning pain he may experience from walking, sunlight, and various other triggers that cause flares for him.
Ascanio Guerriero, 9 years old, was diagnosed by chance with EM in 2012. No other family members have EM. It was extremely difficult to get Ascanio officially diagnosed because there are only five reported cases of EM in Italy and no specialized doctor. Life was absolute hell for four straight years. Ascanio could not wear shoes or enter the kitchen when his mother was cooking. He stayed indoors with the air conditioning running during the hotter months. He spent many nights awake and crying with ice packs resting on his hands and feet. Ascanio is fortunate in that mexiletine has put and kept his EM in remission. He started taking the medication in late 2016. The only real problem has been the side effects from the medication, particularly headaches. His headaches cause him a few sleeping difficulties and concentration problems.
Chandler Keller, 17 years old, was originally diagnosed with Complex Regional Pain Syndrome (CRPS) when she was 12 years old. However, her diagnosis was questioned after a nurse witnessed one of Chandler’s flares while she was recovering from surgery in the hospital. It was during her flare when the nurse mentioned that it resembled EM more than CRPS. The EM diagnosis was later confirmed and Chandler also tested positive for the mutated sodium channel gene. Some days the pain is unbearable for Chandler. Her EM has progressed from her lower legs and feet to her hands and face. She cannot relieve the burning without the assistance of a cold bath or soaking her feet in a bucket of ice water. Because of the agony, sleep is always a challenge. Chandler’s physician put her on gabapentin and she slowly worked her way up to 2,700 mg a day. She has tried almost anything and everything to relieve her pain such as CBD oil, hemp root salve, and several other natural remedies. Her next plan is to try an all-natural whole foods diet. Not a single day has gone by since Chandler was 12 years old where her feet have not been bright red. Her ankles are always swollen. Sadly, her family has learned more about EM from Facebook group forums than from her doctors.
11-year-old Chloe Louise Kuschert was just two years old when her health began to rapidly deteriorate. As her condition progressed, the burning became so excruciating that Chloe could no longer function. Her life became overshadowed by throbbing pain and discomfort. Chloe has a rare disease called Erythromelalgia (EM) which affects her face, ears, and hands. The condition affects 1.3 in 100,000 people, and there is no cure. It causes unbearable burning pain, noticeable redness of the skin, swelling, and increased skin temperature. The exact underlying cause of EM is unknown. It’s thought to result from vasomotor abnormalities or dysfunction in the normal constriction and dilation of the caliber of certain blood vessels, leading to abnormalities of blood flow to the extremities. There isn’t a typical treatment for EM, and sometimes nothing helps. Chloe has tried many prescriptions and supplements, and dealt with dreadful side effects. She is on a strict ‘cooling’ regime to prevent overheating. “Managing her EM pain is incredibly difficult,” said her mother, Jacqui. She went on to say that every day is a battle in which Chloe fights for her joy and freedom from pain. Just a few months following the EM and IC diagnoses, Chloe developed more unbearable symptoms. Her joints began aching and swelling, and she bruised more easily. She had trouble walking due to the pain in her knees and ankles. Ultimately a rheumatologist diagnosed Chloe with Ehlers-Danlos Syndrome (EDS), a group of inherited disorders marked by extremely loose joints, very stretchy skin that bruises easily, and easily damaged blood vessels. EDS affects about 1 in 5,000 people globally. The effects of having all three conditions has caused Chloe to have fainting spells, abdomen pain almost daily, dizziness, and low blood pressure. EM causes the body to quickly overheat during physical activity so Chloe is unable to participate in any outdoor sports.
READ MORE STORIES HERE.
Could medication that helps erythromelalgia also help neurogenic rosacea perhaps?
Below I look into a couple of registered or mentioned EM treatments, and whether or not they could be potentially helpful for subtype 1 or neurogenic rosacea. I also look into public patient testimonials about such trials. But what comes up time and time again, is that UNFORTUNATELY not one treatment works for everyone... Some people mention success with one treatment, where others with similar symptoms see no effect or see their rosacea worsen even. This is highly frustrating. But it is the case for every single rosacea treatment so far, from oral and topical antibiotics to anti-flushing medication to anti-inflammatory medication, antihistamines, anti-pain meds, creams and even supplements. It is trial and error. I tried almost every single treatment option over the years for my own flushing and burning. Some things helped, but most treatments did nothing or made matters worse. I am so tired and deflated by now, that the thought of yet another trial and error is no longer appealing anymore. So I just stick to my anti-flushing medication and have been focusing on just living life with the restrictions that I have now. But trial and error ultimately is the only way to find out if treatments work for us, at this stage in time when there is no cause found for our rosacea and no cure either. The following treatment options have been mentioned for Erythromelalgia flushing, burning and redness of the skin. Some of them have also been suggested sometimes to rosacea patients. I will look into a couple of them as potential rosacea treatment.
-Antiarrhythmics, such as lidocaine and oral mexiletine
Mexiletine is a non-selective voltage-gated sodium channel blocker, which belongs to the Class IB antiarrhythmic group of medicines. It is used to treat arrhythmias within the heart or seriously irregular heartbeats. This is a rarely given off-label treatment. Lidocaine infusions help stabilize pain. You can read more about the use of lidocaine for rosacea here, here and here. You can read more about mexiletine for rosacea here. This young man has erythromelalgia and had success with lidocaine IV infusions:
-Aspirin, pentoxifylline, nifedipine and amlodipine
Aspirin 0.1, twice a day. Pentoxifylline dilates blood vessels for better oxygen delivery to the muscles, eases muscle pain. Nifedipine is a calcium channel blocker (usually contraindicated for rosacea): it works by relaxing the muscles of your heart and blood vessels, dilating them. Used for angina, high blood pressure, Raynaud's phenomenon. Amlodipine is also a calcium channel blocker. You can read more about aspirin for rosacea here, here, here, here and here.
-NSAID's and blood vessel constricting medication
I have written a lot about blood vessel constricting medication, for instance clonidine, propranolol and antidepressants here. I also know from one person with severe erythromelalgia, that a topical clonidine patch made matters worse. The clonidine cream was making the non burning parts of the face painfully cold, whilst the burning parts were unaffected. Later it started to irritate my skin.
-Anticonvulsant drugs like Neurontin (gabapentin) and Lyrica (pregabalin)
I used Neurontin myself for a little while and wrote about it here. You can also read more on Neurontin for rosacea here, here, here and here. Gabapentin is developed to treat nerve related pain. Lyrica (pregabalin) is also used to treat nerve related pain, and is approved for use in diabetic neuropathic pain, and for the use in fibromyalgia. You can also read more on Lyrica for rosacea here, here, here, here, here, here and here. INFO about gabapentin and reduction of hot flushes: https://www.medscape.com/viewarticle/772249
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1627210/
https://www.drugs.com/comments/gabapentin/for-hot-flashes.html
-Certain antidepressants like Cymbalta (duloxetine) and Paroxetine (10 mg daily)
In 2008 Duloxetine became the second drug approved by the FDA to treat fibromyalgia. Used to treat treat depression and generalized anxiety disorder, Duloxetine is also used to treat diabetic neuropathy. Duloxetine is class of medications called selective serotonin and norepinephrine reuptake inhibitors (SNRIs).
-(temporary) Nerve block
This is a very interesting one and I will write a separate blog post in the next month about sympathetic nerve blocks for rosacea flushing and burning, as well as ETS.
Ketamine 0.5% and Amitriptyline 1% in a Lipoderm cream used for Erythromelalgia. It is supposed to help with burning pain and also with the redness. Below is a long medical paper, with highlights of the essential points and the interesting bits from a rosacea point of view. You can also scroll down to the reports from rosacea patients who tried this Ketamine 0.5% and Amitriptyline 1% cream for themselves. When I saw my own dermatologist, professor Anthony Chu, last summer, he mentioned this topical cream to me and said several of his (bad flushing) patients with rosacea saw good results of it. But that it was a slight nightmare to find a pharmacist who was willing to prepare this cream from scratch. But that I should really look into it and find out if I can try out this cream for my own severe flushing and face burning.
Ascanio Guerriero, 9 years old, was diagnosed by chance with EM in 2012. No other family members have EM. It was extremely difficult to get Ascanio officially diagnosed because there are only five reported cases of EM in Italy and no specialized doctor. Life was absolute hell for four straight years. Ascanio could not wear shoes or enter the kitchen when his mother was cooking. He stayed indoors with the air conditioning running during the hotter months. He spent many nights awake and crying with ice packs resting on his hands and feet. Ascanio is fortunate in that mexiletine has put and kept his EM in remission. He started taking the medication in late 2016. The only real problem has been the side effects from the medication, particularly headaches. His headaches cause him a few sleeping difficulties and concentration problems.
Chandler Keller, 17 years old, was originally diagnosed with Complex Regional Pain Syndrome (CRPS) when she was 12 years old. However, her diagnosis was questioned after a nurse witnessed one of Chandler’s flares while she was recovering from surgery in the hospital. It was during her flare when the nurse mentioned that it resembled EM more than CRPS. The EM diagnosis was later confirmed and Chandler also tested positive for the mutated sodium channel gene. Some days the pain is unbearable for Chandler. Her EM has progressed from her lower legs and feet to her hands and face. She cannot relieve the burning without the assistance of a cold bath or soaking her feet in a bucket of ice water. Because of the agony, sleep is always a challenge. Chandler’s physician put her on gabapentin and she slowly worked her way up to 2,700 mg a day. She has tried almost anything and everything to relieve her pain such as CBD oil, hemp root salve, and several other natural remedies. Her next plan is to try an all-natural whole foods diet. Not a single day has gone by since Chandler was 12 years old where her feet have not been bright red. Her ankles are always swollen. Sadly, her family has learned more about EM from Facebook group forums than from her doctors.READ MORE STORIES HERE.
To read more about Erythromelalgia, please see the following blog: Burning for Hope
Could medication that helps erythromelalgia also help neurogenic rosacea perhaps?Below I look into a couple of registered or mentioned EM treatments, and whether or not they could be potentially helpful for subtype 1 or neurogenic rosacea. I also look into public patient testimonials about such trials. But what comes up time and time again, is that UNFORTUNATELY not one treatment works for everyone... Some people mention success with one treatment, where others with similar symptoms see no effect or see their rosacea worsen even. This is highly frustrating. But it is the case for every single rosacea treatment so far, from oral and topical antibiotics to anti-flushing medication to anti-inflammatory medication, antihistamines, anti-pain meds, creams and even supplements. It is trial and error. I tried almost every single treatment option over the years for my own flushing and burning. Some things helped, but most treatments did nothing or made matters worse. I am so tired and deflated by now, that the thought of yet another trial and error is no longer appealing anymore. So I just stick to my anti-flushing medication and have been focusing on just living life with the restrictions that I have now. But trial and error ultimately is the only way to find out if treatments work for us, at this stage in time when there is no cause found for our rosacea and no cure either. The following treatment options have been mentioned for Erythromelalgia flushing, burning and redness of the skin. Some of them have also been suggested sometimes to rosacea patients. I will look into a couple of them as potential rosacea treatment.
-Antiarrhythmics, such as lidocaine and oral mexiletine
Mexiletine is a non-selective voltage-gated sodium channel blocker, which belongs to the Class IB antiarrhythmic group of medicines. It is used to treat arrhythmias within the heart or seriously irregular heartbeats. This is a rarely given off-label treatment. Lidocaine infusions help stabilize pain. You can read more about the use of lidocaine for rosacea here, here and here. You can read more about mexiletine for rosacea here. This young man has erythromelalgia and had success with lidocaine IV infusions:
-Aspirin, pentoxifylline, nifedipine and amlodipineAspirin 0.1, twice a day. Pentoxifylline dilates blood vessels for better oxygen delivery to the muscles, eases muscle pain. Nifedipine is a calcium channel blocker (usually contraindicated for rosacea): it works by relaxing the muscles of your heart and blood vessels, dilating them. Used for angina, high blood pressure, Raynaud's phenomenon. Amlodipine is also a calcium channel blocker. You can read more about aspirin for rosacea here, here, here, here and here.
-NSAID's and blood vessel constricting medication
I have written a lot about blood vessel constricting medication, for instance clonidine, propranolol and antidepressants here. I also know from one person with severe erythromelalgia, that a topical clonidine patch made matters worse. The clonidine cream was making the non burning parts of the face painfully cold, whilst the burning parts were unaffected. Later it started to irritate my skin.
-Anticonvulsant drugs like Neurontin (gabapentin) and Lyrica (pregabalin)
I used Neurontin myself for a little while and wrote about it here. You can also read more on Neurontin for rosacea here, here, here and here. Gabapentin is developed to treat nerve related pain. Lyrica (pregabalin) is also used to treat nerve related pain, and is approved for use in diabetic neuropathic pain, and for the use in fibromyalgia. You can also read more on Lyrica for rosacea here, here, here, here, here, here and here. INFO about gabapentin and reduction of hot flushes: https://www.medscape.com/viewarticle/772249
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1627210/
https://www.drugs.com/comments/gabapentin/for-hot-flashes.html
-Certain antidepressants like Cymbalta (duloxetine) and Paroxetine (10 mg daily)
In 2008 Duloxetine became the second drug approved by the FDA to treat fibromyalgia. Used to treat treat depression and generalized anxiety disorder, Duloxetine is also used to treat diabetic neuropathy. Duloxetine is class of medications called selective serotonin and norepinephrine reuptake inhibitors (SNRIs).
-(temporary) Nerve block
This is a very interesting one and I will write a separate blog post in the next month about sympathetic nerve blocks for rosacea flushing and burning, as well as ETS.
Ketamine 0.5% and Amitriptyline 1% in a Lipoderm cream used for Erythromelalgia. It is supposed to help with burning pain and also with the redness. Below is a long medical paper, with highlights of the essential points and the interesting bits from a rosacea point of view. You can also scroll down to the reports from rosacea patients who tried this Ketamine 0.5% and Amitriptyline 1% cream for themselves. When I saw my own dermatologist, professor Anthony Chu, last summer, he mentioned this topical cream to me and said several of his (bad flushing) patients with rosacea saw good results of it. But that it was a slight nightmare to find a pharmacist who was willing to prepare this cream from scratch. But that I should really look into it and find out if I can try out this cream for my own severe flushing and face burning.
Combination Gel of 1% Amitriptyline and 0.5% Ketamine to Treat Refractory Erythromelalgia Pain. A New Treatment Option?
[STUDY] "A 17-year-old white woman presented with a 2-year history of increasingly frequent episodes of erythromelalgia involving her hands, feet, and lower legs (Figure). She described the discomfort as “throbbing, burning, stinging.” Her symptoms occurred daily. The episodes involved her feet about 10 to 15 times during the day and involved her hands slightly less frequently. Whenever her feet got warm at night, erythromelalgia developed. These episodes were extremely painful. Generally, the erythema and pain occurred independently in the hands and feet. The symptoms were precipitated in her hands and feet by exercise; for example, walking precipitated the symptoms in her feet and legs, and writing precipitated them in her hands. Occasionally, the symptoms occurred when she was at rest, and they occasionally were worse at night. The symptoms lasted from minutes to up to 2 hours. She relieved the symptoms by cooling the affected area with ice and by raising the symptomatic limbs above the level of her heart. At night, she would stick her feet out of bed to relieve the symptoms and, during warm weather, used a fan."
"The symptoms markedly affected the patient's lifestyle. Because the symptoms flared with any activity, she avoided many daily activities, including going to the gym. When she went to a shopping mall, painful episodes developed after a short time, and she would have to sit down or walk very slowly. She missed days at school because of the symptoms. She had no underlying disease or diagnosis apart from attention-deficit/hyperactivity disorder, for which she had been receiving treatment with methylphenidate hydrochloride for the past 10 years. There was no family history of erythromelalgia. Vascular laboratory testing documented the erythromelalgia by demonstrating the expected rise in temperature, which increased from a mean of 18.9°C in her toes without symptoms to approximately 23°C to 24°C with symptoms. This increase occurred concomitantly with bright redness of the skin and a marked increase in blood flow as measured with laser Doppler ultrasonography. In the upper extremities, the temperature was low (21°C) in her fingers during the asymptomatic stage but increased to approximately 33°C with symptoms; there was increased blood flow, similar to that in her toes, without a notable change in transcutaneous oximetry measurements. No evidence of underlying disease was discovered, particularly no evidence of myeloproliferative disease. Although the antinuclear antibody titer (ANA) was increased, the patient had no sign of an underlying connective tissue disease. The results of neurologic investigations, including assessment of large- and small-fiber nerves with electromyography, nerve conduction studies, and autonomic reflex screen (quantitative sudomotor axon reflex testing) were within reference ranges. The erythromelalgia did not respond to treatment with aspirin or misoprostol. Although gabapentin therapy improved the symptoms slightly, it made the patient dizzy. Lidocaine patches had been quite helpful (she wore them all night and before exercising), but many of her normal daily activities continued to be severely curtailed. She gave up all sports at school and stopped attending dancing class. The temperature inside her house was kept between 70°F and 72°F. When she arrived home, she changed into shorts. She wore sandals only, no shoes or socks. At night, she avoided covering her legs with a blanket. She preferred to walk on cold tile floors. When she wrote, the erythromelalgia occasionally became worse, and this interfered with her schoolwork. Her mother photocopied articles that we provided on erythromelalgia and gave them to her daughter's teachers. Because of the erythromelalgia, the patient was given extended time for school examinations."
"Treatment of erythromelalgia is difficult and often unsuccessful. For this young woman, we wanted a treatment that would help control the symptoms and allow her to return to her normal daily activities, namely, to be able to wear shoes and socks, attend the high school formal dance, sleep at night, and participate in school activities. We preferred to avoid systemic medication if possible. We prescribed a combination of topical 1% amitriptyline hydrochloride and 0.5% ketamine hydrochloride to be applied up to 4 or 5 times daily. Two days after this treatment was initiated, the patient reported “spectacular improvement” in her symptoms after applying the combination medication twice daily. She had slept through the night for the first time in 2 years. She had walked through a large shopping mall for 8 hours with her mother. She and her mother were thrilled with the response and said it was the best medication she had tried. They estimated that the symptoms had improved 90%. At follow-up 2 months later, the patient reported that, before going to school, she applied the gel to her hands and feet. This permitted her to engage in the normal activities of the school day. She was able to write and walk without pain. Her episodes of erythromelalgia occurred with approximately the same frequency, but she was able to tolerate them. She has been cautious about returning to gym class but plans to do so. She attended her high school formal dance and wore shoes but did kick them off later in the evening because the auditorium had a cold tile floor. Pain associated with erythromelalgia may be difficult to treat. Because erythromelalgia is not a single disease but a syndrome, the response to various treatments may differ depending on the underlying condition and pathophysiology. Furthermore, treatment of the pain symptoms may have little if any effect on the other manifestations (ie, the redness and increased temperature of the affected limbs). Patient response to erythromelalgia therapy is notoriously variable. Some patients experience long-term pain control with topical agents such as lidocaine, oral anti-inflammatory agents, prostaglandin (misoprostol), vasoactive agents, neuromodulating drugs (ie, antidepressants, anticonvulsants, or antiarrhythmics), or opioids, whereas in other patients the disease is extraordinarily refractory to all measures. [..]
Irritable nociceptors are likely involved in the maintenance of pain in erythromelalgia. The extreme sensitiveness of the skin (ie, allodynia) in these patients clearly supports this concept. However, it does not explain the complexity of the pain, which most likely involves central sensitization. Recent evidence indicates that altered hyperexcitable sodium channels are expressed in inherited painful neuropathies and inherited erythromelalgia. Consequently, we attempted to treat other patients with a topical local anesthetic and had varying success. Amitriptyline, a first-generation tricyclic antidepressant, works by inhibiting serotonin and noradrenaline reuptake; it also blocks sodium channels. Ketamine is an N-methyl-D-aspartate (NMDA) receptor antagonist. The NMDA receptors have a key role in the maintenance of chronic pain syndromes. Normally, after a single painful stimulus, only glutamatergic α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors are activated. However, when a barrage of impulses reaches the dorsal horn, the neuron does not have time to repolarize properly. The NMDA receptors have a magnesium molecule that normally blocks the channel, but when the intracellular potential rises and the neuron remains depolarized for a prolonged period (as with rapid glutamatergic firing), the magnesium molecule is dislodged, thus opening the channel to the influx of calcium and further depolarizing the cell. The influx of calcium activates second messengers and promotes the transcription of various genes, resulting in the increased production of glutamate and other excitatory neurotransmitters and the expression of supersensitive subtypes of sodium channels in primary sensory neurons. This cascade of events leads to increased excitability of the neurons of the pain pathways; thus, painful stimulation continues (the wind-up phenomenon). We speculate that topical application of the combination gel of 1% amitriptyline and 0.5% ketamine works by numerous mechanisms:
1. Ketamine blocks NMDA receptors located on the peripheral terminals of primary nociceptive afferents.10
2. Ketamine may be taken up by the nerve terminals and transported orthodromically to the dorsal root ganglia and dorsal horn. Thus, NMDA receptors at both sites can be blocked.
3. Although ketamine is primarily an NMDA antagonist, it may be able to modulate other glutamatergic receptors, AMPA and kainate, further reducing the discharges from primary nociceptive afferents.
4. Sodium channel blockade by amitriptyline prevents excessive nociceptor discharge by blocking the action potential, similar to a local anesthetic such as lidocaine (which is more potent than amitriptyline). Furthermore, there is evidence that sodium channels are involved in modulating NMDA receptors; thus, amitriptyline too could possibly act indirectly on NMDA receptors.
Although we expected no effect on the other manifestations of erythromelalgia, specifically the redness and high temperature of the affected limbs, 2 patients noticed improvement in those symptoms also, possibly through a decrease in neurogenic inflammation or modulation of efferent nerve fibers to skin vessels (or both). Thus, our topical treatment is a desirable alternative for erythromelalgia symptoms refractory to other treatment. (Source)
You can read more about treatment for neuropathic pain and about nerve blocks and ETS in this blog post.
"Just wondering if anyone else has experience with face and neck flushes? I seem to now also be getting very red cheeks, face and neck. Comes and goes. Also red ears. (This is on top of the usual feet/hands burning). If you do, is there anything you used that helped cleared it up? [..] Like tonight for example - very cold night. Feet are fine but cheeks are burning... Thanks in advance, Craig"
Mads replied in October 2013: "Hi Ocker, Yes, I get flushes or flaring of face, neck, ears and scalp. My EM started in my right knee, then feet etc.working its way up my body rather like a fungus spreading.. I had very acute onset (2-3 months), which has now remained completely widespread and chronic(8 months now). I'm struggling. Really struggling! Most research states EM is progressive. To help minimize - very difficult and very much trial/error. Depends what you respond to - all of us need an individualized poly-pharmacy approach. I responded moderately to lidocaine so 'theoretically' my EM is linked to sodium channel blockers or analogs of such like mexiletine. Other folk respond to serotonin antagonists. I have listed things that i have tried with/without luck in my profile section. This is an awful thing isn't it. Do you know what type of EM you might have? Mine is primary, idiopathic, God bless. Please pm me if you wish to offload/chat about symptoms etc. Hoping you have a 'comfortable' night as possible. Mads"
Clarissajo replied in October 2013: "Ocker, I too have this occur frequently.... EM can hit me in the face, ears, and chest as well as arms, hands, feet, legs. Unfortunately I don't know anything about pharmaceuticals for EM, my doctors have only suggested aspirin, which I cannot take for other reasons. I'm interested in learning more about possible meds for this condition. I do keep feet elevated and try to stay as cool as possible. [..] I currently take Lyrica (pregabalin) which has similar effects. I didn't realize that it was so similar to gabapentin until very recently.... apparently a lot of people take either gabapentin OR lyrica, and usually choose gabapentin because it's less expensive. My neurologist is prescribing the Lyrica plus Cymbalta because that combo is very effective against my fibromyalgia pain, and also she says the Lyrica helps prevent seizures which I have a tendency to have. Thanks again!Take care, JoAnn"
Gaga replied in October 2013: "Mine started with my face and ears. I had that for about 2 years. I was sure I must have Lupus due to other symptoms but doctors just said I had red face syndrome and red ear syndrome. The ears are so touchy sore that just my hair touching the tops of my ears feels like my ears are raw skin. When my cheeks get real bright which is every day, several times and sometimes constantly for days, I look like a clown. I get so explosively hot and my head sweats too. My feet started about 2 years ago. My feet also go numb due to Spinal Stenosis but they are sore. My big toes and the foot just behind the big toe hurt so bad and are so sore. The soles of my feet are deep red even when the hot feeling eases off. The only help I've had with my feet is to rub with a menthol liniment to give a false cool feeling and then turn a fan on them. Wetting my head with cool water sometimes eases the warmth of my face but not my ears. Today it felt like the heat was attacking my spine. This is just awful."
LibbyK replied in October 2013: "I've been having persistent redness and occasional episodes of heat in my face - mostly nose and upper cheeks. We thought it could be lupus but my ANA test was fine. I suspect it may be rosacea with an EM flare here and there. I've been using only products for sensitive skin (helps a little as I do have dry, sensitive skin) and wearing makeup to cover the red when I go out. I haven't tried any drugs."
Mary replied in October 2013: "I’ve had neck and facial flushing for over three years now. There seems to be no way that I know of to get rid of the flushing. It does come and go. What I do take daily to help for the pain is Gabapentin…it works great for me!!! Now when I was in my 30’s and I got Rosacea I used Metrogel that I got from my Dermatologist and I still use it and that helps a tiny bit for burning along with medication."Alina_Delp replied in December 2013: "Hi Craig. I too have flushing in the face and chest but I don’t like to call it flushing I like to call it burning! It only comes with warmth like my EM and only goes when cooled. At first the doctors thought it was just a skin rash like rosacea but it burns like a bad sun burn and very hot to the touch. Like Gaga said just a gentle touch like a hair touching it hurts. Ouch! Take ace and stay cool. Alina"
Flaming replied in August 2015: "I have burning in my neck and face. Started about eight months ago. It is very bad at night. I keep a cooler beside my bed and use a gel lack wrapped in a dish towel to cool my face. I tried aspirin when I was first diagnosed and it helped a bit when it I took it at 4 am when the pain was at its worse. I could only tolerate (does was super high: 1200 mg’s) for five night before my stomach pain put a stop to that. I have secondary EM and my doctor hopes that this short trial with aspirin shows that I could benefit from lidocaine infusion. I hope to get that in the Fall. It was my pain doctor who diagnosed me. I think rosacea and EM are very different, so shouldn’t be hard to differentiate for a medical doc. My pain doc isn’t an Em expert, but she could diagnose it. My GP thought it was hot flashes from menopause. Even I knew that was not the case. Hot flashes (and rosacea) do not hurt!!! {Scarlet Nat: Incorrect; neuropathic rosacea does hurt}. I see a neurologist today who is supposedly the only person in town (Vancouver) who knows anything about EM. Here’s hoping she can help. First, I have to get the dang EMG testing over with prior to the consult today. If I have anything helpful for to report, I will post, for sure. Take care, all."
And here is another medical article on this topic: Is Topical Ketamine Ready For Prime Time?In summary, the authors state that ketamine is used for general anesthesia, and as a topical cream has been used for the management of neuropathic (nerve) pain. Like in the above article, it is explained how ketamine works on a cellular level. For now, people rely on their own pharmacist to make this ketamine cream, in a neutral carrier base. Topical ketamine strengths range from 0.5% to 10% in published studies, with the majority of studies using from 0.5% to 2.0%. There are very few studies at higher doses. It has mixed to positive reviews for the treatment of postherpic neuralgia (which also gives nerve pain of the skin). It showed less improvement in patients with diabetic neuropathy. Ketamine combined with amitriptyline in a topical cream was also more effective than a placebo, in trials. Topical ketamine cream did reduce pain in patients with Complex Regional Pain Syndrome. Studies showed that topical ketamine is safe and that topical ketamine with as high a ketamine level of 10%, did not result in any systemic absorption into the blood stream and body, and appeared safe. It is probably not wise to make a cream with a higher strength of ketamine. Possible side effects are itching, mild burning, and nausea.
Laser_cat sent me this very interesting link to a "heat desensitizing protocol" for EM patients:
It is interesting that EM has this "heat desensitizing protocol" has similarities to the "Warm room theory" for rosacea (read more about that one here).
In this Reddit post, someone with rosacea / erythromelalgia posted about success with Amitriptyline 1% and Ketamine 0.5% cream
Despotic_Monarch wrote in 2017: "Dealing with Rosacea/Erythromelalgia for two years. Hi! Erythromelalgia is a Neurovascular condition with very similar aspects to Rosacea but is many times worse. The only thing out of 25 or so drugs/medications that worked is a cream base that contains... Amitriptyline 1.0%/ Ketamine 0.5% cream in a 1 to 1 ratio. In just one week I went from being a tomato to a piece of paper and finally my skin can heal. If you are suffering from Rosacea this might be an amazing/safe treatment for you! Please see your derm. Study First picture is before use of the cream (around the starting 3pm flush). Please note I had to edit the coloring because I don't have an exact picture for this time period. Second picture is after the cream (No more flushing). I guess my condition was so bad that not even suicidal doses of prednisone wouldn't reduce the redness. Gotta keep fighting and never give up!"Boywonder95 replied: "Can you comment on whether or not it's still working for you please? I'm looking into the process on how to go about getting it in the states. It looks like I have to convince my doctor/ or derm to get this specially made at a compound pharmacy."
Despotic_Monarch replied: "Its amazing I DON'T EVEN HAVE TO USE IT TWICE A DAY. Just twice a week or so. Trust me I had the worst Erythromelalgia that you could possibly get. I'm 100% clear now. No side effects. Show him this post and my pics." The author also shared his story on The Rosacea Forum, here.
There is also a lengthy thread on The Rosacea Forum about the (trial) use of Amitriptyline/ Ketamine cream. I selected the most interesting posts here:Deansm wrote on December 2nd, 2016: "Cleared up my type 1 Rosacea (flushing red nose). I have had flushing and swelling (caused by the flushing) on my nose for around 7 years (no pustules or pustules). Tried antibiotics, Soolantra, metrogel etc, nothing worked, every treatment has had absolutely zero effect. Two months ago the GP I am seeing prescribed me an ointment containing Ketamine 0.5% and Amitriptyline 1%. Within 3 days of using it twice a day the flushing disappeared and my skin is white, the same as it was before Rosacea. It has been the same ever since for the last two months. I can eat, exercise, go out in the sun and nothing will bring on a flush. If anyone has this subtype and nothing has worked have a look into this, I have no side effects (the GP said it is very safe as the amount of active ingredients is too low and it's not absorbed systemically). I am not sure which or if both of the ingredients are working. I have since learned that topical Ketamine behaves as an anti-inflammatory by acting on Toll-like receptor (TLRs), which leads to down regulation of proinflammatory gene expression. The packaging has no branding, it was made in a compounding pharmacy. He said he has found it helpful going back years for patients with Erythromelalgia and it was worth trying for my Rosacea as nothing else worked."
Hg24 replied on December 3rd, 2016: "This is really interesting, Dean. I googled and found reference to a study about this topical for erythromelalgia. The ointment seems to be for the pain (versus redness). I have type 1 rosacea, too. Flushing and permanent redness. Dean, you mentioned that your skin color is back to normal (pre-rosacea). So did you also have permanent redness? Or just the redness that comes on temporarily when you flushed? I guess I'm wondering if the ointment returned your vessels to normal - constricted them back. I wonder if the ingredients work because they address nerve pain. Nerves can stimulate our flushing. Eg, nerves release acetylcholine, a chemical that tells our vessels to dilate."
Deansm wrote on December 3rd, 2016: "It probably is completely up to the GP, how many treatments you have already tried etc. He told me the ointment reduced redness and flushing on patients with Erythromelalgia (no idea what this was). I never expected this to work as every other treatment he prescribed (Soolantra, Antibiotics, Metrogel) has either done nothing or even made my Rosacea worse. My Rosacea started (I was 24) as a sudden huge flareup (huge flushing episode) on my nose when I went to bed one night. I had never blushed or flushed on my nose before that first flareup. Ever since every night without fail I would have a flareup when I went to bed. Through the day I had one or two flareups and my nose was sensitive to flushing but not as bad as in the night. After each flare up it would take around 2-3 hours for my skin to return normalish in colour but there was always a slight pink hue on my nose. The worse symptom of all is the nose swelling for around 7 hours when I sleep and waking up every morning with a swollen nose. The flushing has stopped now and that stopped the swelling. My nose still looks a bit swollen but it's slowly going down now (2 months so far)."
Fiugs replied on December 4th, 2016: "There is a topical with the brand name Epicept, although I think the compound's percentages are different than you describe, Deansm. Did your GP indicate if s/he thought this would be a short or long term solution? Just wondering because I got the impression that some topical analgesics lose their effectiveness after a while.... I say that only based on what my local pharmacist told me in relation to a lidocaine based gel I was inquiring about; he said it would stop working after a while. I will certainly ask my GP about this! [..] I also found this article, referring to a compound using the same percentages as Deansm has been given. Where about in a scotland are you, Skullmcrex - and do you know of a decent derm? I'm still trying to find one that I feel in genuinely interested in trying to help. Thanks Deansm and all the best."
Deansm replied on December 4th, 2016: "From looking at the link EpiCept Cream is much stronger (2% ketamine / 4% amitriptyline), The cream I am on is 1% amitriptyline and 0.5% ketamine and it stops the flushing (the lower dose it works the better). Since my doctor said he had success with the ointment on patients with Erythromelalgia I have been reading a lot on Erythromelalgia forums about the safety of this cream (it seems very safe at low dose). I am not sure which ingredient (or a combination of both) is stopping the flushing, but this is something I found about topical ketamine. Also from reading various Erythromelalgia forum posts, the vasodilation, flushing, redness are very similar to Rosacea, only it happens on the feet and hands and sometimes the face in Erythromelalgia. The only real difference over type 1 Rosacea as far as symptoms is the addition of more pain, stinging. It is interesting hearing about the other treatments they use on those forums to stop flushing that might also work for flushing on people with Rosacea."
Brady Barrows replied on December 4th, 2016: "Flugs, What a wonderful find to substantiate this thread. Note what the article says: "Erythromelalgia is a clinical syndrome characterized by attacks in which the affected limbs become bright red, hot, and excruciatingly painful. The common trigger is heat exposure induced by exercise or increased ambient temperature. As the condition progresses, the limbs become permanently red, hot, and painful. The pain of erythromelalgia, commonly described as hot and burning in quality, is frequently disabling, and patients obtain relief by lowering skin temperature, for example, by applying cold objects to the affected area, exposing the affected extremities to cold surfaces, immersing the limb in ice water, or by elevating the affected extremity. Frequently, these measures damage the skin, further exacerbating the pain, and occasionally cause maceration. Pain associated with erythromelalgia may be difficult to treat. Because erythromelalgia is not a single disease but a syndrome, the response to various treatments may differ depending on the underlying condition and pathophysiology. Furthermore, treatment of the pain symptoms may have little if any effect on the other manifestations (ie, the redness and increased temperature of the affected limbs). According to various theories, erythromelalgia is a vascular disorder or neuropathic condition. In previous studies, we have shown that both systems are involved, but we do not know which one is affected first. It is plausible that the vascular and neuropathic components differ in each subject depending on the underlying cause." - Hopefully in the future we will know more if this treatment for erythromelalgia will be a standard treatment for rosacea."Arsenalista replied on December 5th, 2016: "Looks like another guy has also great results with this stuff.. I read about Erythromelalgia (first time hear about it) and it looks like something very similar to rosacea but more painful. One of my worst symptoms is a chronic burning feeling at all my face. Damn, have to get the prescription."
I replied on December 6th, 2016: "A very interesting find! I'm very curious if more people with facial (rosacea) flushing and burning will find relief from this cream. It's also a big bonus if your pharmacist can just add the powdered medication to your regular moisturizer. It's a fascinating distinction between facial erythromelalgia and rosacea. I'm not completely sure how you can detect one from the other, (apart from EM typically at the least affecting the hands and feet - but people with rosacea can get Raynaud's (me too), which can mimic these symptoms a lot), as these cases mentioned here look and sound pretty much like rosacea.
"A 53-year-old man presented to an outside dermatologist with an erythematous (skin redness) facial eruption of 4 years' duration. The eruption was associated with burning and swelling, which the patient reported worsened with sun exposure. These symptoms were debilitating, preventing the patient from working and maintaining daily activities. Initially, the eruption was thought to be rosacea and actinic damage (pre-cancer of the skin); however, it was unresponsive to standard therapies." "Upon further questioning, the patient reported skin burning not only with sun exposure, but also with heat exposure. Furthermore, he reported attempting cooling measures, such as the frequent use of ice packs, to relieve his symptoms. With these additional details, a clinical diagnosis of facial and auricular erythromelalgia was established. Further workup excluded potential causes of secondary erythromelalgia. Given that erythromelalgia is thought to involve both small fiber neuropathy as well as vasculopathy, we initiated therapy with aspirin, pentoxifylline, and
gabapentin. Subsequently, nifedipine was added to this treatment regimen. At 5 months' follow-up, the patient reported 80% improvement and was able to return to work and enjoy
normal daily activities."
I am curious if aspirin, pentoxifylline and gabapentin together could help some of us too. And this woman seems to have serious rosacea, and she wonders whether or not she has erythromelalgia or rosacea. And people with FIBROMYALGIA also mention rosacea symptoms more often, here for instance. A nice person with fybro emailed me that her rosacea disappeared once she started to take guaifenesin tablets (a decongestant). Anyway, shows how complex these skin conditions are, there might be a lot of overlap and different factors at play. So curious to hear who else here might have benefit from this cream that is suggested."
Hg24 replied on December 8th, 2016: "This is interesting: In the video description, the patients says the only thing that's helped with her particular condition is a cream with amitriptyline, gabapentin, clonidine and ketamine. This is a link to an article that mentions the compound cream. As I saw elsewhere, it says the cream is used for pain. I'm just wondering how that translates to flushing and redness. If our nerves stimulate our blood vessels, maybe the cream has the potential to quiet any nerve impulses. Ketamine appears to have an anesthetic component. The doc in this article mentions a side effect of nightmares, which he says is a known side effect of ketamine. Also, he mentions that it is absorbed systemically. He only prescribes it for small parts of the body for this reason." (Watch HERE on youtube)
Brady Barrows replied on December 9th, 2016: "The cool item in this article you found (excellent find hg24!) is that it reports, "Of more than 1,000 Mayo Clinic patients who have tried the combination, "I’d say less than 1% has told me that they’ve ever had a side effect," he said. "I’ve had just two or three patients tell me they’ve gotten nightmares," a known effect of ketamine. "This is a product that has great promise," he noted." So we have 1000 patients who have tried this out. It is a wonder that rosacea sufferers haven't heard about this until now."BVokey replied on December 9th, 2016: "I found this article which may provide some insight into the question on translation to flushing and redness. This is one of the articles I will be sharing with my derm. It references the Mayo Clinic study on this cream and notes at the end of paragraph four of the introduction: "In addition, ketamine has been hypothesized to act through a number of pathways, including binding of receptors for N-methyl-D-aspartate, α-amino-3-hydroxy-5-methyl-4-isoxazole propionate, and kainate. These medications may act synergistically to limit transmission of painful stimuli. In addition, the same biochemical effects may prevent vasodilation, thereby reducing the characteristic redness and warmth of the disorder.
Neuropathy & Neuralgia Therapies
Local Anesthetics, Gabapentin, Ketamine, Amitiptyline
❧ Bind to Na+, K+ and Ca2+ channels in damaged nerves to suppress abnormal spontaneous activity
❧ Disrupt G protein coupling to produce anti- inflammatory effects
❧ Typical usage has been shown to be safe and without serious adverse effects
❧ Consider surface area
PainFree-IBU Fast Gel
• Ketamine 10%.......NMDA Blocker, Analgesic
• Gabapentin 4%..............Nerve Blocker
• Ibuprofen 10%...............Anti inflammatory
• Diclofenac 4%................Anti inflammatory
• Amitriptyline 1%.............Nerve Blocker
• Cyclobenzaprine 2%..... Muscle Relaxant
• Apply 0.5-1.0ml 3-4 x daily
• Use 1-4 drops on the affected area either alone or after the PLO gels. Response is in 1-2 minutes."
Brady Barrows wrote on December 15th, 2016: "The concept is flushing aggravates rosacea usually producing a rosacea flareup. Flushing avoidance is an accepted treatment for rosacea. If this ointment using Ketamine 0.5% and Amitriptyline 1% reduces the flushing, it may also reduce the redness associated with the flush. This ointment may or may not reduce the flushing in all patients but it did for Deansm as he reports in the first post of this thread. In the paper that uses this treatment for EM, the docs were using this ointment to manage the pain and reported, "Amitriptyline, a first-generation tricyclic antidepressant, works by inhibiting serotonin and noradrenaline reuptake; it also blocks sodium channels. Ketamine is an N-methyl-D-aspartate (NMDA) receptor antagonist. The NMDA receptors have a key role in the maintenance of chronic pain syndromes." So until we get more reports, we don't know if this works for other rosacea sufferers or whether it reduces the flushing or the redness or both. Deansm has reported back so we can't find out if it is still working or not for him? Not reporting back may indicate that it is still working for him and he has gone his merry way."BVokey updated on December 27th, 2016: "A quick update to everyone here. I developed a cold over the last 24 hours and have been redder than normal (whenever I get a cold, which is not often, I will always be redder than usual), and then this afternoon I had salad with spinach (I practically always avoid spinach as it causes me to flare up but I thought maybe I would be ok), but sure enough I got very red from the spinach. So this has been a small set back with my progress regarding this cream. I am still planning on using it for a long period of time as overall (aside from this past day) it has been helping tremendously up until I got sick. I will continue to report. Hopefully more people are also able to test this out and report back."
Hg24 wrote on December 28th, 2016: "I wonder if this cream is essentially acting as a numbing cream. Ketamine is an anesthetic. And amitriptyline quiets nerves. BVokey, does the cream cause any sensations on the skin that you've noticed? Does it feel numb? Or anything like sting, itch, feel hot Already BVOKEY is having a different experience than Deansm - in that Dean said he wasn't flushing from his usual triggers - heat, exercise, etc. Whereas BVOKEY, your usual flare-up issues can still provoke some level of reaction, redness. Though it's early days. I also think that rosacea on the nose can be different from rosacea on the delicate skin of the cheeks. Also, the different spatial, vessel, nerve infrastructure that lies underneath nose vs cheeks, chin, forehead."BVokey wrote on December 28th, 2016: "No it doesn't produce any sensation while on my skin. It's not drying and it doesn't feel like anything at all."
BVokey updated on January 2nd, 2017: "I have mixed feelings about it. Early on it was working great but after about 4-5 days it seemed it wasn't doing much. I feel like whenever something starts to work, my body combats it so that it stops working. I do think it is helping somewhat with my baseline redness but it doesn't help with preventing a flush. It certainly isn't making things worse. I will still post here if things change around, however now I just moved onto msm and dao supplements to see if they are helping. Good luck to you in getting this compound from your doctor. If anyone else with type 1 Rosacea gets relief from this compound please share your results. [..] I began using the compound 2x daily (morning and evening) on the 22nd and it significantly reduced the redness for maybe 4-5 days and since then it seems it hasn't been helping as much. I'm still using it twice a day and plan to continue using for at least a couple months. Along with the compound cream, I was taking 5000iu vitamin d3 (I was deficient when I tested last year and now my levels are normal but I still continue it daily), and Metamucil fiber supplements. I had been taking these two supplements way before starting the compound cream. I still take the d3 and fiber. Since the compound creams' effects have seemed to not be helping as much anymore, I added msm (3g daily) and the enzyme Diamine Oxidase (aka DAO which helps break down histamine, to find out if a lack of this enzyme is responsible for my issues with foods containing histamine). I also just started on propranolol 10mg 1x daily due to my GPs recommendation. So in total I take d3, fiber, msm, dao, and propranolol, and put the compound on my face twice a day. If the cream had continued to work as significantly as it was working early on, I would have just kept with it without adding anything else to the mix. The only issue with this is if I get relief, its going to be more difficult to tell what did it. [..] I don't have proof that it works - I was more inclined to post a photo if it cured my redness. It hasn't. I think it is helping somewhat, but it's tough to say what helps as I am taking several things at the moment but something's definitely helping significantly with my baseline redness. It helped significantly for a few days, then not so much, then I moved on to a few different supplements and now my skin is doing great. However I still have to avoid my triggers such as alcohol, dairy which is the biggest one, along with a host of other foods like spinach, eggplant, vinegar, avocado, etc. If I slip up, it makes no difference what I'm doing to help my skin, I will still flare up which could last a couple days before my skin calms down, which is why it is no cure for me. Here are some photos to show how my baseline redness has improved basically since starting the ointment. First photo taken literally moments before first application of the ointment and second was three days into using the ointment where my skin was at its best, and third photo was taken two days ago, a little after three weeks since starting this. My baseline has never been so clear and I've had permanent redness for around 15 years. But again, I can still flare which will throw things off. I think the ointment is definitely contributing to the improvement, but it's likely also be a combination of several things I am taking. As far as the drug industry not catching up with the science, this ointment is in practice for erythromelalgia, not Rosacea. As far as I know, the medical journals don't have anything tying this to rosacea."
Skullmcrex wrote on January 20th, 2017: "Maybe we all have erythromelalgia and the rosacea is a mental condition. Prozac has been working quite well for my Rosacea (Soolantra being doing stuff too). Hell I'm eating whatever I want to counteract some pretty significant weight loss and I'm not noticing any major worsening of symptoms. The ketamine thing is funny because it's a really popular recreational drug here, I've seen many people on it recently (utterly moronic drug to take and that's coming from a pretty liberal drug user, I essentially watched people become paraplegics/trapped in their own bodies making zombie like noises and muscle spasms) I think every rosacea sufferer should try anti depressants and a good physical/semi physical suncream. I think that's the most important thing honestly. The diet thing seems to be less of a factor personally, although it does somewhat increase minor acne for me. Avoiding tetracyclines that increase light sensitivity in the skin significantly hence worsening classic rosacea symptoms is also important. [..] Antihistamines seems to help a lot too, especially at dinner time (I don't get the dreaded evening flushes anymore). Basically everyone on here should take anti depressants. (hope you're all well)"
Laser_cat wrote on January 24th, 2017: "Hi, I hope the trouble with finding compounding pharmacies is worked out soon! I got my cream (it's very thick and goopy) and used it yesterday morning, yesterday night, and this morning. Actually, mine is 10% ketamine / 2% amitriptyline. Although I didn't have an immediate reaction, I noticed about 10-20 min after applying, I had a mild flush (more of a blushing, nothing compared to my regular evening flushes), which lasted 5 or 10 min. That scared me a little, but I'll def. stick with it for a while. Last evening, there were a few patches on my face that flushed and burned, but most of my face seemed ok. Maybe I didn't put it on evenly enough? Not sure. My general problem is flushing evening / night. Interestingly, I tried putting it on my ears too yesterday morning, and yesterday evening they flushed real bad. So I won't put it on my ears anymore, see how that goes. I'll update later again. ~laser_cat (Lizzy). PS - It cost me 50 $ (US) for 1 month supply (my insurance didn't cover)"Brady Barrows wrote on January 25th, 2017: "Lizzy, Why did your physician prescribe 10% ketamine / 2% amitriptyline when Deansm reports the Rx is Ketamine 0.5% and Amitriptyline 1% (see the first post in this thread)? This is radically a different prescription."
Laser_cat wrote on January 25th, 2017: "Hi Brady, Not sure why 10%. I didn't mention the anecdotal report of Deansm. I just suggested the ket/am topical combo. My rheum says he has experience with flushing and vasomotor abnormalities (and was the first doc not to give me metrogel/aczone/sulfur washes etc). I didn't see it was 10% until the pharm gave it to me. Anyway, after I applied it yesterday morning, at 3 pm (my evening flushing usually happens at least 2 hours later) my face started to burn/flush in patches, in the order of where I applied it. Until my entire face was burning so bad (the worst since I started clonidine months ago). I am not sure if it was due to the ketamine 10%, something specific to me personally not being able to tolerate the cream, or what. Actually, my face is a bit sore today when I washed with water / put on moisturizer. That was surprising, since I've read that this combo is usually well-tolerated (although it may or may not give results). I'll try to ask my rheum why the 10% (seems like even in EM papers, it is much less than 10%). In the meantime, my rheum wants me to go from 0.3 mg clonidine/day to 0.2 mg/day (slowly), to raise my blood pressure enough so I could try a calcium channel blocker (typical for EM, a blood vessel relaxer in hopes to prevent their spasming). That idea is very scary/risky, but I have nothing to lose really, my symptoms are so debilitating right now and have already tried so many things that did nothing/made things worse. (Actually, I know a couple ppl are helped by magnesium on here which is similar to a CCB.) Sorry, I wound up writing a novel. Lizzy."
Brady Barrows wrote on January 25th, 2017: "Rosaceans like novels, so no problem. Your Rx seems extreme compared to clinical papers that use the 1% Amitriptyline and 0.5% Ketamine Rx"
Fiugs wrote on January 26th, 2017: "I now have the ointment. So I collected my ointment yesterday... at last! The timing is unfortunate, as I had my 3rd IPL session the day before yesterday and my face tends to through a few days of greater volatility after that. Because of this, I decided it would make sense to wait for another couple of days before using the ointment, otherwise how could I/we know what was effecting me, either positively or otherwise.. So... Last night I went and applied the ointment.... I know... I know... I just couldn't resist. I initially decided that there was probably little point in my reporting the results of last night's application - and I wouldn't have bothered, except for the fact that they are similar to yours, Laser Cat ... All day yesterday, my post IPL face was a little less red, lovely and cool and flush free. Within an hour after applying the ointment, the most reactive part of my face (left cheek) flushed quite badly. That was around 9.30.... I woke briefly at 1.30 am and observed that it was still flushed. I will add that, unlike some other users' reports here, I did experience a few sensations upon application. They were very mild however, not painful or even uncomfortable. A slight tingling on the cheeks and a feeling of warmth, although, at that pre-flush stage my face did not feel warm to the touch at all. Now... The caveat to all of the above is that my typical reaction, post IPL, is pretty much what I have described above and it usually kicks in a just over 24 hours after an IPL. So, given the timing, this event could just have been that, rather than a reaction to the ointment. The worthwhile point to note, I guess, is that the ointment did not prevent the flush... and of course it is unclear at this point whether or not it caused it.
John I asked my pharmacist at boots in Stirling, Scotland if they could speculate as to why you were unable to get your presc fulfilled at Boots in the Republic or in N.I .they said there could be two reasons -
1 - The republic, and each of the UK's devolved parliaments, will have their own medical regulations with regard to prescriptions involving controlled drugs, such as Ketamine.
2 - My local Boots said they had to work quite hard to find one of the few special pharmacies in the UK that were able to source and handle such drugs. They speculated that the ones you have tried, John, may not have been willing - or able - to give it that much time and attention. The good news for you (I hope) is that my pharm did tell me which special pharmacy was able to do the job: they are called IPS Specials and are located in Surrey, England. Here is a link to it.
I can tell you now that you will not be able to send your presc direct to them in order to get it fulfilled... But perhaps if you give their contact details to your pharm, they won't have to do the legwork for you and might be able to help you more. I would encourage you to do your best to get a prescription that specifies Lipoderm, or some other easily absorbable base. I'll post again when there is more to share. All the best, folks."
Kalamazoo wrote on January 29th, 2017: "Noteworthy success with Amitriptyline (25mg) Thought I would add my experience. I have had rosacea for around twenty years. The flushing blushing/nose swelling type for almost the entire period. Now, some blemishes too. Two years ago I started flushing like mad. I had recently come off 50mg doxycycline (which was really destroying my digestive system). I had no blemishes then just extreme flushing, usually in the evening. Once I flushed so badly that my neck and chest area flushed bright red too (this only happened once but it was extremely distressing and painful). I had recently moved to a new country and so I was feeling really challenged in every way possible. High stress levels. A couple of years before a doc had recommended Amitriptyline hydrochloride (a derivative of Amitriptyline) to me as she thought maybe I had fibromyalgia. This was just after my mom had died and I was a wreck, emotionally and physically. I had gone to see that doctor because I had strange aches and pains, numbness and ice cold feet. I took it for a limited time at 10mg. Anyway, after all these bad flushes, I decided to ask a doctor to prescribe Amitriptyline for me at 25mg. It was a fairly random decision. I am still taking it now, two years later. My skin improved A LOT quite quickly and the flushing almost completely stopped. My skin wasn´t perfect but I was able to enjoy a relatively normal life, even to start running again. Other factors that could have influenced the improvement: I was eating healthily (no junk, no processed food, low-ish carbs), I had cut down on but not eliminated dairy, I had reduced caffeine, I started taking vitamin D 5000iu two or three times a week, and I started doing regular yoga to help with my stress levels. I really think the thing that did the trick was the Amitriptyline though. For the whole two years since I started taking it my skin has more of less behaved until now (a different story on a different post (recent) about an experiment gone wrong). Sigh. It certainly didn't cure my rosacea but it controlled it to an acceptable level. Amitriptyline is usually used to treat depression, anxiety, fibromyalgia, chronic pain etc. As an anti-depressant it is prescribed in much higher doses than the 25mg that I was taking. According to Wikipedia it's 'exact mechanism of action is unclear'. Interestingly, when a family member got shingles a few years ago, he was prescribed Amitriptyline. Apparently it switches off pain at the root of the nerve."
BVokey wrote on February 2nd, 2017: "I'm still doing very well and haven't had this much relief in my life. However the ointment strength I am using is the same as what the OP posted, nothing higher as I'm reading some people have way too high strengths for the ketamine. I'm also taking care of myself and avoiding my triggers, the biggest trigger being anything dairy (even if a bagel or bun has any milk ingredients I won't eat it or I will flare). I am also taking propranolol and MSM supplements and maybe they are helping as well. But it's no cure I still have Rosacea it's just much more manageable than ever before, and my face can still flare if I don't be careful with my food and environmental triggers."BVokey wrote on February 16th, 2017: "I've been using this ointment for a couple of months now and I haven't rebounded once. I tried mirvaso awhile ago which gave me severe rebound after a few hours. I wouldn't be concerned with this causing any rebound effect but since everyone's body is so individual, if symptoms get worse probably best to stop. The only thing that causes any flushing for me anymore is diet related. This ointment I think has helped, but there could be several factors at play. It definitely hasn't caused any harm."
BVokey updated on February 22nd, 2017: "Still using 2x daily. It's going very well. I did not use it for one day over the recent weekend to see if it would matter, and my face was significantly more red that day. It's helpful for sure but it will not prevent me from a flush from food triggers. It does not eliminate my redness completely and I still have a way to go before I'm rosacea free."
Vice replied on February 23rd, 2017: "Nobody should be worried about ketamine having an effect. I take ketamine in powdered form recreationally and in my eyes 'medicinally'. It has an antidepressant effect and is currently being reviewed and researched as a breakthrough anti depressant for treatment resistant patients. It's a wonder drug really. The hallucinations are more distortions and only occur with high doses. - I personally love them myself. I am also trying to get the cream ointment and am in the middle of the process to getting it in uk now but it's difficult. Obviously I know it won't have any effect on my mind but it's still crazy that a drug I used systemically to get high might also be a good treatment for Rosacea! Who'd have thought!"
Fiugs replied on February 25th, 2017: "So far it seems these are four sum results with this topical:
Deansm - only has Rosacea symptoms on his nose. He reported good results.. But then disappeared from the forum, so we do not know if the ointment continues to help. (Is it fair to speculate that his Rosacea was at a moderate stage if it was confined to his nose?)
Fiugs - unable to tolerate the ointment on my face. It produces a burning sensation and my face goes red within minutes as a result. Irritation lasts for at least 2-3 hours.
Laser_Cat - similar results to Fiugs: note that the % active ingredients in Laser_cat's ointment was much higher than the others.
BVokey - reports good results. No reported irritation upon application. Says his skin is better than its been for a long time. However, he began several other treatments around the same time, inc DAO and Propranolol and says he cannot be sure which is helping."
Nobrakes wrote on February 28th, 2017: "For you guys with erythromelalgia like symptoms, I too had the same thing. For me taking flaxseed oil seems to have miraculously placed it into remission, though I'm not sure why"
RaymondS wrote on April 14th, 2017: "I'm on the 3rd day and no change in my rosacea. It's making my skin really oily and I think this morning it's slowly starting to aggravate it. Will keep trying but defiantly no calming effect so far. Didn't harm me but didn't help either. And it was a hell of a process to get the stuff. Just proves what works for one doesn't always work for another. I will keep it and just use in one area to make sure that over time it works or doesn't. But unfortunately it did not cure me."
Fiugs wrote on April 8th, 2017: "I gave up on mine quite some time ago as it really aggravated my skin. It seems that only one person on our forum - the original poster - got marked results with this. It's truly weird, isn't it, that our skins are all so different! Every day I grow more of the opinion that there is no such single condition as Rosacea, but rather it's a blanket term for a red, irritated and sensitive flushy face, that may or may not include P&Ps, the unique cure of which is probably only going to be determined by the cause(s)."Laser_cat wrote on December 16th, 2017: "In case anyone made it this far in the thread: I have a contact w/ EM who said to me ketamine/amitriptyline combo cream produced a heating effect a little while after application (as some ppl in this thread mention ... happened to me as well). He tried the ketamine cream alone which didn't have this heating effect, and found that topical ketamine was as effective for pain as a (temporary) nerve block. I'm retrying the combo cream (1% ketamine, 2% amitriptyline) for the past few days. It's been 3 days. Sometimes I experience the initial heating effect (for me, happens 1 hour after application, lasts for ~1 hour) andsometimes not. After, my face feels numb, supported, generally good. I'm hoping to get separate creams at some point containing a single active ingredient to see which one will be the most helpful. ( FWIW many things can be compounded as topicals -- clonidine, diclofenac, baclofen, etc eg ttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5340828/
After poking around reading about pain, "wind up", EM, etc, I think for anyone experiencing lots of burning/pain during flushing, that it is important to get that controlled somehow ASAP (if it exists after beta blockers, NSAID's, etc) because some of these mechanisms seem to become irreversible over time ( eg, https://courses.washington.edu/conj/sensory/pain.htm ) I know for EM specifically there is thought to be a "golden window" of opportunity in some patients where the pain is still reversible before it becomes irreversible." [..] FWIW my impression is there is little difference between neurogenic (burning) rosacea and facial EM. The treatments for both are trial and error and involve the same set of drugs pretty much. I know my doctor has prescribed ketamine / amitriptyline gels for neurogenic rosacea (as well as topical gabapentin, topical clonidine, topical NSAID's, etc).
I think ideally someone would get ketamine gel alone AND amitriptyline gel alone and maybe can mix them in a 1:1 ratio if there is no problem with each. I am trying the combo cream right now, so cannot say if the ketamine gel alone (or amitriptyline gel alone) would be better. From the paper I linked above (written by world's experts in EM) they seem to think the ketamine + amitriptyline would act synergistically. But my impression from the EM forums is that ketamine gel alone might be sufficient. Sorry I can't say anymore. [..] I still think it might be best to get the lowest concentration of ketamine and amitriptyline individually (so you can use 1 or both if you want). But I think ketamine is the ingredient responsible for the "heating effect" that I observed. I have amitriptyline 10% + flurbiprofen 1 % (the latter is an NSAID) and don't have noticeable heating. I have tried ketamine 10% + amitripytline 2% in the past and it had a major heating effect. Now I use the amitriptyline 10% + flurbiprofen 1% combo or amitryptiline 0.5% + ketamine 0.5% combo. (The flurbiprofen helps my nose swelling flushing actually ... which makes sense since ibuprofen helps my nose but unfortunately not my cheeks.) I talked to the compounding pharm and he said ketamine is the one mostly responsible for any heating. I also talked to another derm and he said the same thing."
The more I read and try to write here about other flushing disorders especially about erythromelalgia (EM), the more I start to think that this extreme face flushing and burning might not be strictly 'rosacea'. I feel there is a grey zone, where bad neurogenic/ vascular rosacea falls into, as well as EM, as well as perhaps bad flushing from neuropathic pain disorders. The more I hear and read, the more it seems that it is extreme to flush this bad. And the few people I know who have it too, all seem to balance on the edge of different possible diagnosis. For instance, one of the derms I see thinks I have a mast cell disorder, hence the violent flushing. I also know a bad flusher who's doctor says she must have erythromelalgia. I joined an EM facebook group and there are many more people there with extreme face flushing and burning! More than in your average rosacea group. However they tend to also have burning hands and feet. My hands do burn but that has been diagnosed as Raynaud's syndrome in hospital. But the division lines are blurry and I might just as well have EM instead of straight forward rosacea. But... at the end of the day, they are labels and EM doctors also struggle to treat the facial burning and flushing, so we end up either way with a flushing problem that is hard to treat; both for dermatologists as well as for neurologists as well as for a rheumatologist, or a vascular specialist...
