tag:blogger.com,1999:blog-27786329490143651212024-03-13T21:08:27.949-07:00Red Man SyndromeErythroderma, Exfoliative Dermatitis and Related Skin Conditions. Plus a Rash of Dark Humour!Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.comBlogger8125tag:blogger.com,1999:blog-2778632949014365121.post-31557802892390662812012-04-16T21:55:00.001-07:002012-04-17T20:42:11.685-07:00PresentationThis section presents all the characteristics of classic erythroderma. It will look at the range of physical symptoms as well as physiological changes that may appear symptomless but which manifest in diagnostic tests. It is especially important to understand these ‘hidden symptoms’ and know how to manage them before they ever start to manifest as physical symptoms.<br />
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Let me add here that symptoms, causes and personal history are inextricably linked. Your number one diagnostic tool is a notebook and pen! It is important you keep a diary of how your skin condition started, when it manifested and how it is developing. Memory is notoriously fallible, so best to write everything down. Think hard about what may have triggered the skin eruption. Had you taken any drugs? Some drugs have long half-lives so go back a couple of months or more. Any contact with potentially toxic chemicals? Our seas, for example, have unfortunately turned into open dumping grounds and may contain a cocktail of toxins and microorganisms. Do you have any family members who suffer from a skin condition, even if a mild one? It is good to inform your dermatologist of any genetic link to a potentially underlying condition.<br />
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Once armed with your notebook, you can proceed to attempt a self-diagnosis with the knowledge that a list of symptoms is not enough; a true differential diagnosis involves both positive data as well as negative data. If you want to see how it’s done, then watch House MD, and see how often Dr Gregory House gets it wrong before he hits on the correct diagnosis.<br />
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v 1.0Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.com0tag:blogger.com,1999:blog-2778632949014365121.post-31548577675530435342012-04-16T20:58:00.001-07:002012-04-16T21:21:55.438-07:00Red Man Syndrome and l'homme rouge<u>Red Man Syndrome</u><br />
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Red Man Syndrome proper is now used to describe an erythroderma that remains idiopathic. The word 'idiopathic' is a wonderful medical term that means 'of unknown cause', or 'we haven't a clue'. You may find it of interest to read my Diary of a Red Man blog entry on idiopathy so that it is clear what we mean by the question, “What is the cause of your erythroderma?”<br />
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One problem with defining Red Man Syndrome in terms of a lack of known causes is that, if one day your dermatologist actually finds the root cause of your condition, then the name of your condition will change too; all without any apparent change in symptoms. However, the most important thing is that with the new name comes a new treatment. It is worth remembering that all names are just labels and that it is more important to know what each label means rather than being attached to a mere name.<br />
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However, Red Man Syndrome also has a number of characteristic symptoms: palmoplantar keratoderma (a hardening of the skin on the palms of the hands and soles of the feet); dermatopathic lymphadenopathy (a problem with the lymphatic system requiring a lymph node biopsy for verification); and a raised level of serum immunoglobulin E (IgE), which requires a blood test to verify. <br />
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<u>L’homme rouge</u><br />
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<i>L’homme rouge</i> is, of course, merely the French for ‘red man’, but now (perhaps confusingly) refers to an erythroderma that is secondary to cutaneous T-cell lymphoma (CTCL). Other types of exfoliative dermatitis that have been brought into the secondary CTCL camp are mycosis fungoides, Wilson-Brocq, Hebra and Savill types. You may still find these names on the internet but their reclassification is a significant step forward in the often obscure etiology of this skin condition.<br />
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v 1.0Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.com0tag:blogger.com,1999:blog-2778632949014365121.post-71510390750488483762012-04-16T20:24:00.002-07:002012-04-16T21:23:08.351-07:00PathophysiologyPathophysiology refers to how the nature of a disease (its pathology) manifests through changes in bodily functions (or physiology). Sometimes these changes express themselves as symptoms but often they appear symptomless to the patient and only manifest in blood test results and other diagnostic techniques. Skin conditions have a complex pathology and often affect physiology in unexpected ways. <br />
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We have already seen in the overview that the most obvious symptoms of erythroderma are the red skin rashes which can expand to the whole body and which start to exfoliate within a few days. Let us now look at these additional pathophysiological symptoms and how to manage them. As always, specific advice from your personal dermatologist overrides any general advice given here; your personal biology and the medications you are on will affect symptoms and the management of your condition.<br />
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<a href="http://redmansyndrome.blogspot.com/2012/04/homeostatic-dysregulation.html">Homeostatic Dysregulation</a>. Your body’s autopilot is malfunctioning; you need to take control of your body’s temperature regulation.<br />
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<a href="http://redmansyndrome.blogspot.com/2012/04/albumin-loss-eat-more-proteins.html">Albumin Loss</a>. Shedding skin means a loss of proteins, especially albumin; eat more proteins.<br />
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v 1.0Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.com0tag:blogger.com,1999:blog-2778632949014365121.post-13642243046995192832012-04-16T19:57:00.001-07:002012-04-16T21:24:01.775-07:00Homeostatic DysregulationThe redness of the skin is due to an increase in blood supply to the capillaries. When a layer of skin is shed, the new skin surface sometimes looks almost like normal skin, but unfortunately will return to the previous shade of red within a few hours. However, the real problem is not the redness itself but that the skin is dry and has lost the ability to sweat properly; the result is a dysfunction of the body’s normal temperature control and of its natural electrolyte balance.<br />
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Temperature dysregulation is a very serious problem, resulting in heat loss, water loss and possible hypothermia. The normal mechanism to regulate the body’s heat and water through sweating no longer seems to function, instead the skin transpires, which is a physical process like the evaporation of water from wet clothes and not under direct control. <br />
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You may sometimes feel that the erythematous part of the skin is getting hot. The heat sensors in the skin send a message to the brain which sends a signal to the heart to increase the flow of blood. This is the normal reaction to cool down the body. Unfortunately, your body is not really overheating, your temperature sensors are sending the wrong information. The natural response is, again, to keep pumping more blood to cool down the body. This can lead to a serious positive feedback loop in which your heart rate and blood pressure may rise to dangerous levels. Cardiac failure is a real possibility here. The good news is that the body has other temperature sensors, such as in the normal skin you still have as well as other locations. Eventually the brain reconciles the conflicting information by siding with the body’s core temperature readings. Your heart rate will start to drop and this episode of homeostatic dysfunction will be over. <br />
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There are a few things you can do to minimize these attacks. Firstly, if you are in the middle of doing something, stop it! Find a temperate environment, lie down, relax and breathe slowly and deeply like in meditation and help the heart slow down. Slow breathing also conflicts with the idea that your body is doing some form of exercise and should, therefore, be breathing more quickly; this also helps the brain discount the false temperature signals it is receiving. <br />
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If you are in a hot environment, head for an air-conditioned room; if you are in a cold place then go to somewhere warmer. Both temperature extremes are to be avoided if at all possible. If you were not doing anything physically taxing then just relax and breathe slowly till your heart slows down too. It is also worth having a thermometer close to hand. You can quickly verify that your hot flush was a dysfunction by measuring your core temperature. You should find that your core temperature is normal. However, if it is high then you should investigate whether you are suffering from a secondary infection and the start of a fever. As an aside, it is worth measuring your temperature on a daily basis as you may find that your ‘normal’ temperature is marginally higher than it used to be. This is yet another minor symptom and worth knowing what is your new baseline.<br />
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The final point to be made here is that the larger the area of red skin the greater the level of dysregulation. People who have not yet been diagnosed with erythroderma are most probably totally unaware of these problems. It is easy to walk around for days with a torso covered in erythroderma because the initial symptoms seem so mild. If you have a red rash that is expanding, go see a dermatologist as soon as possible. It may not be erythroderma, but it’s also not likely to disappear on its own.<br />
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v 1.0Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.com0tag:blogger.com,1999:blog-2778632949014365121.post-63192665304902675502012-04-16T19:39:00.002-07:002012-04-16T21:24:46.801-07:00Albumin Loss: Eat More ProteinsA defining characteristic of exfoliative dermatitis is, obviously, exfoliation. The first wave of skin loss happens about 2-6 days after the start of the first patch of erythema. The skin may peel off in small sheets; don’t pull it off as your skin is thinning and you may be prone to cracks and bleeding. Indeed, one clue to a possible underlying cause of erythroderma is that the skin is growing too quickly compared to its normal rate of growth. It is also shedding too quickly, a process that is usually imperceptible to most of us. This peeling is probably associated with a protein known as CEA and we shall look at this more closely in the section on etiology.<br />
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For now, it is important to be aware that the body may be shedding about 20-30 g of skin per day, compared with about 8 g per day of normal loss. This results in an excessive loss of serum albumin, the most common blood plasma protein in the human body, and a condition known as hypoalbuminemia. Albumin is only synthesized in the liver, at the rate of about 12-25 g per day, and the normal rate of loss tends to balance this, giving a stable total of 250-500 g of albumin in humans. The average person loses about 14 g of albumin per day through a variety of mechanisms, with about 3-4 g lost through exfoliating skin. So, if the skin is shedding at 3 or 4 times its normal rate then you need to replenish about 10-15 g of albumin. <br />
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In theory, this is easily achieved by adding two to four extra eggs per day. However, this is only true if you eat them raw as cooking will denature a part of the albumin in the egg whites. This is also true with other high-protein foods that need cooking, such as meats and fish. Perhaps the best sources are dairy products, peanuts and tofu. The issue is complex and I shall investigate this further. The key thing now is that you need to increase your protein intake to compensate for the extra loss of albumin. This will be discussed at length in the Nutrition section.<br />
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v 1.0Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.com0tag:blogger.com,1999:blog-2778632949014365121.post-41466571980711648752012-04-13T20:22:00.005-07:002012-04-17T20:43:34.876-07:00Erythroderma Overview<u>Erythroderma</u><br />
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Erythroderma is also known as Generalized Exfoliative Dermatitis. The word 'erythroderma' is derived from the Greek words <i>erythros </i>(meaning red) and <i>derma </i>(skin). In the early stages of the condition, you may hear your doctor mention the word 'erythema' as a description (which is just the medical term for redness) before a firm diagnosis can be established. It is also common that erythroderma develops from a different initial skin condition.<br />
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The basic symptoms of erythroderma are peeling red skin covering 90% or more of the skin surface. One problem with this definition is that once the redness has reached 90% it is already a very serious condition. It is therefore important to consult an experienced dermatologist as early as possible. Skin diseases have a habit of transforming themselves from one condition into another. Nobody yet really understands how or why they do this, but it is important that you, as a patient, monitor your initial skin rash for any changes. <br />
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Not only are skin conditions often difficult to diagnose in their early stages, but their underlying cause may only become clear as the disease progresses. This is especially true with erythroderma, where data indicates that some 30% of cases remain idiopathic, or without known cause, even if the treatment has worked and the condition gone into remission. This means that your dermatologist will have a list of possible skin conditions and possible causes in his mind and will advise you on the most likely diagnosis at the time of the consultation. One of those possibilities may be erythroderma but it will only rise to the top of the list if and when the symptoms warrant it.<br />
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Having said all the above, let me reiterate that the first aim of this whole Red Man Syndrome blog is to guide you as much as possible towards a valid differential diagnosis; the symptoms you do not have are often as important as those that you do. The second aim of this blog is to give the best possible advice on how to treat and manage the condition for those with a confirmed erythroderma.<br />
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Erythroderma is a fairly rare condition but it is also a very dangerous condition to be left untreated. It is unlikely that your general doctor will have experience of the subtle differences between skin conditions, so it is important to see a dermatologist as soon as possible.<br />
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v 1.2Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.com0tag:blogger.com,1999:blog-2778632949014365121.post-58998576640189769612012-04-08T05:18:00.002-07:002012-04-08T23:53:17.793-07:00Diary of a Red Man: The Ginger Red ManSitting in the back of a Bangkok taxi, my little daughter decides to entertain me with her latest sing-song.<br />
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“Run, run, run as fast as you can;<br />
You can't catch me, I'm the Gingerbread Man!”<br />
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She then looks at me and says, “Daddy, you are the Ginger <i>Red </i>Man!”<br />
That girl needs an agent. She looks pleased with her little pun and launches into a reprise:<br />
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“Run, run, run as fast as you can;<br />
You can't catch me, I'm the Ginger Red Man!”<br />
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She laughs and gives me a big hug, then looks down at my exfoliating zombie hand, “I like red!” She could charm the skin off a snake.<br />
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Now, I knew that this was originally a long song but, try as I might, was unable to dredge up such an esoteric and banal memory. After a bit of strenuous research, here is a modern adaptation of the story.<br />
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<div class="separator" style="clear: both; text-align: center;"><a href="http://2.bp.blogspot.com/-yEdrjJ84LcE/T4F98FdPXVI/AAAAAAAAANE/c8d3d4qh3SM/s1600/ginger-red-man.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="200" src="http://2.bp.blogspot.com/-yEdrjJ84LcE/T4F98FdPXVI/AAAAAAAAANE/c8d3d4qh3SM/s200/ginger-red-man.jpg" width="168" /></a></div><br />
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There was once a little old man and a little old woman, who lived in a little old house in the edge of a wood. They would have been a very happy old couple but for one thing: they had no little child, and they wished for one very much. One day, when the little old woman was baking gingerbread, she cut a cake in the shape of a little boy, and put it into the oven.<br />
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Presently she went to the oven to see if it was baked. As soon as the oven door was opened, the little gingerbread boy jumped out, and began to run away as fast as he could go.<br />
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The little old woman called her husband, and they both ran after him. But they could not catch him. And soon the gingerbread boy came to a barn full of threshers. He called out to them as he went by, saying:<br />
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“I've run away from a little old woman,<br />
A little old man,<br />
And I can run away from you, I can!<br />
Run, run, run as fast as you can;<br />
You can't catch me, I'm the Gingerbread Man!”<br />
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The annoying little ingrate continued to pester the local simpletons and the gullible farm animals, taunting them with his silly little song. But he was right, he was quick. With his pursuers trailling a fair distance behind, the gingerbread boy eventually came across a fox.<br />
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“I've run away from a little old woman,<br />
A little old man,<br />
And I can . . .”<br />
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“Yes, I know.” interjected the fox. “Looks like thirsty work to me. Here’s a little drink to help keep your speed up. You see, if you keep those dumb animals running they will be exhausted soon and easy prey for my supper.” The gingerbread boy thought this was a great idea and gulped down the drink. The other animals were getting close enough to bait again.<br />
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“Run, run, run as fast as you can;<br />
You can't ...”<br />
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The gingerbread boy suddenly looked down at himself in horror. "Oh dear! I'm a quarter <i>red</i>!" And then, "Oh, I'm half red!" And soon, "I'm three-quarters red!" The gingerbread boy was having a serious allergic reaction to the fox’s potion, finally saying, "I'm <i>all </i>red!" <br />
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Sensing he was in danger of being caught the gingerbread boy yelled:<br />
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“Run, run, run as fast as you can;<br />
You can't catch me, I'm the Ginger Red Man!”<br />
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He promptly fell over, just like a limp soggy biscuit would do. The fox lapped him up and scarpered.<br />
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<div class="separator" style="clear: both; text-align: center;"></div><div class="separator" style="clear: both; text-align: center;"><a href="http://4.bp.blogspot.com/-A9Or56LoSYQ/T4GD1rm5JQI/AAAAAAAAANM/EU7GsZqwVY0/s1600/ginger-red-man2.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="200" src="http://4.bp.blogspot.com/-A9Or56LoSYQ/T4GD1rm5JQI/AAAAAAAAANM/EU7GsZqwVY0/s200/ginger-red-man2.jpg" width="168" /></a></div><br />
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<i>With thanks to <a href="http://www.surlalunefairytales.com/gingerbread/index.html" target="_blank">The Annotated Gingerbread Man</a> by Sur La Lune Fairy Tales</i>Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.com0tag:blogger.com,1999:blog-2778632949014365121.post-71821013358779450642012-04-04T16:43:00.004-07:002012-04-08T23:54:37.604-07:00Diary of a Red Man: Better Red Than Dead<div class="separator" style="clear: both; text-align: center;"><a href="http://3.bp.blogspot.com/-C9UgrgzOj50/T35SyTnLSXI/AAAAAAAAAM8/W8edFsvr7Ts/s1600/128px-Staunender_Mann-rev.jpg" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img alt="By Elmar Ersch (Own work) [CC-BY-SA-3.0 (www.creativecommons.org/licenses/by-sa/3.0)], via Wikimedia Commons" border="0" src="http://3.bp.blogspot.com/-C9UgrgzOj50/T35SyTnLSXI/AAAAAAAAAM8/W8edFsvr7Ts/s1600/128px-Staunender_Mann-rev.jpg" title="By Elmar Ersch (Own work) [CC-BY-SA-3.0 (www.creativecommons.org/licenses/by-sa/3.0)], via Wikimedia Commons" /></a></div>Never before have I seen a doctor so visibly shocked. It was almost cartoon perfect, with hands raised in horror, eyes bursting out of their sockets and only pure professional decorum restrained him from emitting a shriek. “You have to be admitted to hospital... right now!” That was the start of the end of my life.<br />
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I didn’t even have time to gloat over my correct internet-driven self-diagnosis; had to satisfy myself in private later. Just two days prior to this, another dermatologist uncharacteristically admitted to being clueless as to my condition. Yet here was a doctor pushing me onto the critical list. If a misdiagnosis can be fatal, the advantage of a <i>non</i>-diagnosis is the freedom to seek another opinion. Especially when it appears that erythroderma can be fatal.<br />
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No large-scale study has been done on this, but the data emerging suggests that erythroderma has a potential mortality rate of about 40%. Huh?! 40%!! Even my dermatologist plucked this number out of the air when he was castigating me for allowing the internet’s amateur diagnoses to stress me out. However, even Medscape, a website for health professionals, quotes a figure of 43%, although it tempers this alarmist figure by saying that only 18% of these could be linked directly to the skin condition. This actually places the mortality rate at about the 10% mark. With so many high numbers the only conclusion is that erythroderma can kill you!<br />
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I initially had the morbid thought that many cases of erythroderma were diagnosed at the autopsy. I was walking around with this condition for some two or three weeks before someone identified it. Apart from the slowly expanding red rash, my own symptoms lacked any itchiness or soreness, nor were there any pussy infected spots and the exfoliation had not yet started. On the whole, it was perfectly possible to carry on working, hiding the rash beneath a corporate uniform. <br />
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With hindsight, the problem with erythroderma is that it does not ring a loud enough alarm bell as to how lethal it can be if untreated. It needs a corny pun such as “A Red Rash to a Bull!” I know, everyone loves a corny pun. The real problem is that the red skin no longer functions normally in helping the body regulate itself. Your skin is fundamental in regulating the body’s temperature, water and electrolyte balance. As the rash expands so the level of dysfunction increases. There comes a point when the amount of red skin puts the body under risk of a serious malfunction.<br />
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I will look more closely at all the above issues in the first few articles and diary entries. I don’t wholly believe this alarmist 40% mortality rate any more and will review the current medical literature on this. However, the message here is that some kind of alarm bell <i>is </i>necessary. If you have a condition in any way similar to erythroderma you really need to find an experienced dermatologist. Even once you have been diagnosed and are trying to sort out your life, you will need to learn how to control some bodily functions that were previously on automatic. The risk of messing this up is very real.<br />
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I have had to learn a lot very quickly. The aim of this website is to share this with you. It is also important to cover the full range of symptoms so I welcome advice from people with similar conditions but different experiences.<br />
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For better or worse, there’s no stopping on a red route.Anonymoushttp://www.blogger.com/profile/17700702422324131665noreply@blogger.com0