Materiały źródłowe

• #1 Erythromelalgia: Symptoms, Causes, Treatment

  https://my.clevelandclinic.org/health/diseases/22752-erythromelalgia

  There isnt much you can do to prevent erythromelalgia. If you have a family history of the disease, genetic testing can uncover the likelihood of passing it on to a fetus. […] There are many steps you can take to feel your best and ease discomfort. These include: […] Avoiding food and beverage triggers: Pay careful attention to what you eat and drink so that you dont consume items like caffeine or spicy foods that may raise your temperature. […] Exercising: Choose gentle activities that get you moving without significantly raising your temperature. These include swimming, yoga and Tai Chi. […] Staying in cool environments: If you will be outdoors, try to do so in the morning when temperatures are cooler. […] Bathing carefully: Choose showers instead of baths to avoid skin injuries. You can also keep your body cool by opening a window and using lukewarm water. […] Planning ahead: Since symptom flares tend to occur at night, take care of essential tasks earlier in the day. […] Getting organized before sleeping: Keep a fan or portable air conditioning unit near your bed. And use the lightest possible bed sheets or no top sheets at all.

• #2 What Is Erythromelalgia?

  https://www.webmd.com/heart-disease/what-is-erythromelalgia

  Erythromelalgia is not a preventable disease. Still, genetic testing can be done on those with a family history of erythromelalgia to discover whether or not there is a chance of your unborn child ending up with the condition. […] While erythromelalgia is not wholly preventable, there are ways to prevent flare-ups from occurring as frequently and to be more comfortable when they do occur. […] If you wish to prevent flare-ups, consider the following: Avoid certain food and beverages: Caffeinated beverages and spicy foods can increase the temperature of your body, thus triggering an episode of erythromelalgia. Avoiding these drinks and foods can help prevent these episodes from occurring and prevent the symptoms from worsening. […] Adjust your exercises: Intense workouts can cause an increase in body temperature as well. If you plan on exercising, choose gentle activities such as swimming and yoga.

• #3 Erythromelalgia | 5-Minute Clinical Consult

  https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816851/all/Erythromelalgia

  Primary EM cannot be prevented. If due to a known condition, secondary EM improves and recurrences prevented with adequate disease management.

• #4 Erythromelalgia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557787/

  Management of erythromelalgia can be quite challenging and necessitates an interprofessional approach. Treatment should encompass patient education, behavior modifications, and the avoidance of triggers. Various treatment options have been suggested, though none are fully curative, rather aimed at symptom management and improving quality of life. While treatment of erythromelalgia is primarily focused on symptom control, secondary erythromelalgia can improve or resolve with treatment of the underlying disease process. […] The mainstay of therapy for both primary and secondary erythromelalgia aims to avoid triggers, most often heat, exercise, and standing. Commonly used strategies include remaining in cool environments, decreased physical activity, limb elevation, and avoiding excess clothing. In addition, some patients find relief with cool water immersion or portable fans. It should be noted that excessive cooling via ice water immersion can lead to maceration, infection, and ulceration.

• #5 Erythromelalgia: a cutaneous manifestation of neuropathy?* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-erythromelalgia-cutaneous-manifestation-neuropathy-articulo-S0365059620303676

  The prevention of crisis is based on a strict control of triggers and promotion of preventive measures. […] The prevention is based on a strict control of triggering events, i.e. heat exposure or pressure, standing position for a long time, physical activity, emotions, and/or stress, alcohol ingestion, spicy food, vasodilator drugs, night time, exposure to chronic vibration or even light skin contact in severe cases.

• #6 Erythromelalgia | MedLink Neurology

  https://www.medlink.com/articles/erythromelalgia

  Because erythromelalgia is precipitated via exposure to elevated temperatures, strict future avoidance of such environments should be encouraged. For example, exercise regimens that require exposure to arid environments should be avoided. Furthermore, recreational activities such as sauna bathing should be minimized. In cases of secondary erythromelalgia, immediate withdrawal of the offending agent or treatment of underlying conditions is a prerequisite to mitigating symptoms and minimizing the risk of future exacerbation. Cooling the affected area has been shown to minimize pain and associated symptoms. However, patients should be cautioned not to immerse their limbs in ice water, which can cause gangrene and life-threatening hypothermia, but rather to use a fan. […] Medical management is the most effective method of treating primary erythromelalgia and should include genetic counseling and symptomatic management of exacerbations. There have been several case reports, mostly in pediatric cases, demonstrating success in using epidural infusions to treat erythromelalgia pain. A case report has demonstrated success in a 34-year-old woman who received a 3-day infusion of 0.0625% ropivacaine with 2 mcg/ml fentanyl after failing treatment with aspirin. Her pain was reportedly reduced from 10/10 to 0-1/10 during the treatment. On follow-up 9 months after treatment, she reported her pain as 0-2/10. During this time, the ulcers on her lower extremities healed, and erythema resolved.

• #7 SciELO Brazil – Erythromelalgia: a cutaneous manifestation of neuropathy? Erythromelalgia: a cutaneous manifestation of neuropathy?

  https://www.scielo.br/j/abd/a/bFN55FCQkZfpYSNyV6WKY3F/

  The prevention is based on a strict control of triggering events, i.e. heat exposure or pressure, standing position for a long time, physical activity, emotions, and/or stress, alcohol ingestion, spicy food, vasodilator drugs, night time, exposure to chronic vibration or even light skin contact in severe cases. […] Preventive measures should be taken into account. Peripheral vasodilator drugs are contraindicated as well as exposure to excessive warmth, intensive physical exercise, smoking, and physical or psychological stress. Comfortable shoes and appropriate to weather or temperature are indicated, as well as physical rehabilitation and psychic support. Physical exercise without impact 2 or 3 times weekly should be recommended and also yoga and swimming.

• #8 Erythromelalgia (Erythermalgia) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/erythromelalgia-erythermalgia/

  Patients should be followed up constantly because lifelong treatment is necessary, particularly for those with primary erythromelalgia. […] Factors that trigger erythrolmelalgia, such as heat, hot drink, alcohol and spicy food should be avoided. […] Air conditioning and cooling fans are safer than icy water immersion. […] Elevating the affected extremities may relieve symptoms in some cases.

• #9 Erythromelalgia: Symptoms, Causes, Treatment | Qwark

  https://qwarkhealth.com/conditions/erythromelalgia

  Preventative measures for Erythromelalgia include avoiding triggers that can cause symptoms such as hot temperatures, standing for long periods, and emotional stress. […] The risk of Erythromelalgia occurrence can be minimized by identifying triggers and avoiding them. Patients can work with their healthcare provider to develop a personalized plan for managing their symptoms. Additionally, maintaining good circulation through regular exercise and avoiding tight clothing or shoes can help reduce the risk of Erythromelalgia. […] To prevent the onset of Erythromelalgia symptoms, patients should avoid known triggers such as warm temperatures, spicy foods, and alcohol. Wearing comfortable clothing and shoes, and elevating the affected areas can also help to prevent symptoms. Patients should also seek prompt medical attention if they experience any symptoms to prevent further complications.

• #10 What Is Erythromelalgia?

  https://www.webmd.com/heart-disease/what-is-erythromelalgia

  Stay in cool conditions: Its important when dealing with erythromelalgia that you stay in the coolest conditions possible. If you plan on participating in outdoor activities, aim to do so in the morning hours before the temperature rises. […] Be careful when bathing: To avoid skin wounds, choose showers over baths. Also, use lukewarm water and open a window to help you stay cool. […] Be prepared: Flareups occur most commonly in the evening and nighttime hours and can affect your nightly chores and sleep schedule. Instead of waiting until late in the day to do important tasks, complete them in the morning and early afternoon. Additionally, you may want to squeeze in a nap, if possible, in the daytime hours. […] Practice organization before sleeping: Organize the essentials prior to bedtime. Keeping a fan or portable air conditioner next to your bed can help you stay cool. You can also use the lightest bed sheets possible. Alternatively, you can also choose to use no top sheets at all to alleviate symptoms.

• #11 Rare Foot Conditions – Erythromelalgia

  https://balancehealth.com/resources/rare-foot-conditions-erythromelalgia/bay-area-foot-care/

  Erythromelalgia is a rare foot condition affecting the body’s extremities, including the feet. It is a chronic condition and needs lifestyle management and medication for a better quality of life. […] Lifestyle changes can help manage and prevent flare-ups. Some small ways to manage this condition include: […] Avoiding food and beverage triggers like caffeine or spicy foods that may raise the temperature. […] Doing gentle exercises that provide physical activity without significantly raising temperatures, like swimming, yoga, and Tai Chi. […] Staying in cool environments. […] Avoiding going out on very warm days. […] Taking an umbrella while venturing out during the day to protect yourself from direct heat. […] Doing outdoor activities in the evenings when it is cooler. […] Using an air conditioner ensures the body temperature is not too high on warm days.

• #12 Manifestations in the Oral Mucosa of Erythromelalgia. A Case Report

  https://opendentistryjournal.com/VOLUME/13/PAGE/61/FULLTEXT/

  However, the use of common trigger prevention measures, such as avoiding exposure to temperatures above 30C, use of comfortable footwear, performing moderate physical activity and local application of cold proved to be more effective and the patient enjoyed a practically normal quality of life. […] Since there is no effective drug treatment, here we demonstrate that controlling the factors that trigger the pain episodes and together with performing moderate physical activity, comfortable footwear and local application of cold would result in the remission or attenuation of patients symptoms, thereby improving their quality of life.

• #13 Rare Foot Conditions – Erythromelalgia

  https://balancehealth.com/resources/rare-foot-conditions-erythromelalgia/bay-area-foot-care/

  Staying hydrated. […] Wearing soft, light cotton clothes. […] Using the lightest possible bed sheets at night time. […] Using cold water while taking a bath. […] A good podiatrist who is an expert at treating rare foot conditions will be able to devise a treatment plan that can help manage this rare and painful condition.

• #14 Erythromelalgia | MedLink Neurology

  https://www.medlink.com/articles/erythromelalgia

  In secondary erythromelalgia, the key to treatment is the identification of the underlying cause. Treatment of this often mitigates the symptoms and leads to remission of outbreaks. For example, phlebotomy in patients with polycythemia and effectively normalizing platelet counts in patients with essential thrombocytopenia can initiate a commensurate decrease in secondary erythromelalgia symptoms. In cases where an external stimulus such as heat is the suspected exacerbation trigger, strict future avoidance of such stimuli should be recommended. In the event that a certain medication yields an eruption of symptoms, immediate withdrawal of the offending agent is recommended. Low doses of aspirin have been shown to reduce the incidence of both arterial and venous thrombosis, thereby improving symptoms associated with the disorder. Thus, prophylactic use of low-dose aspirin is recommended to all patients with secondary erythromelalgia, particularly those with polycythemia vera-induced erythromelalgia.

• #15 Aspirin cures erythromelalgia and cerebrovascular disturbances in JAK2-thrombocythemia through platelet-cycloxygenase inhibition

  https://www.wjgnet.com/2218-6204/full/v6/i3/32.htm

  Aspirin cures erythromelalgia and cerebrovascular disturbances in JAK2-thrombocythemia through platelet-cycloxygenase inhibition. […] Irreversible platelet cyco-oxygenase inhibition by aspirin cures the erythromelalgia, MIAs and microvascular events, corrects shortened platelet survival to normal, and returns increased plasma levels of beta-TG, platelet factor 4, thrombomoduline and urinary thromboxane B2 excretion to normal in symptomatic JAK2-thrombocythemia patients. […] The complete relief (cure) of burning pain and red congestion by one dose aspirin (350 to 500 mg) for a few days is diagnostic for thrombocythemia in ET and PV patients. […] Reversible inhibtion of platelet COX-1 activity by indomethacin 25 mg TID is an alternative to relief erythromelalgia. […] Aspirin was discontinued in busulfan induced thrombocythemia with normal platelet count in 13 ET and 11 PV patients.

• #16 Aspirin cures erythromelalgia and cerebrovascular disturbances in JAK2-thrombocythemia through platelet-cycloxygenase inhibition

  https://www.wjgnet.com/2218-6204/full/v6/i3/32.htm

  The cure of erythromelalgia by aspirin could be documented in 15 thrombocythemia patients. […] Treatment with a loading dose 350 to 500 mg followed by 100 mg once daily cures erythromelalgia, its acrocyanotic complications as well as the migraine-like atypical TIAs (MIAs) and acute coronary syndromes (ACS) through irreversible inhibition of platelet cyclo-oxygenase.

• #17 Erythromelalgia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557787/

  Approach to the management in steps: (a) Avoid triggers (b) Aspirin for one month (c) Topical drugs for two to four weeks (c) Systemic drugs like gabapentin or pregabalin or venlafaxine for two to four months (d) Consider other systemic drugs and pain rehabilitation programs. […] Patients suffering from both primary and secondary erythromelalgia should receive proper education on the etiology of the disease. Thorough screening and evaluation to rule out myeloproliferative disorders found to be associated with secondary erythromelalgia is a crucial component to the management and prompt diagnosis of underlying disease processes.

• #18 Anyone out there with Erythromelalgia? | Mayo Clinic Connect

  https://connect.mayoclinic.org/discussion/erythromelalgia/

  There is an Erythromelalgia Clinic in Minnesota which serves as a focal point for clinical research at Mayo Clinic. The care at the Erythromelalgia Clinic is guided by a team of doctors, including specialists in dermatology, neurology and vascular medicine. These specialists work together to offer you specialized skin testing, an accurate diagnose and a treatment plan that meets your needs. […] I am searching for new medicine, on Gabapentin now and it does nothing, and researching for a successful treatment! […] I am now on Lyrica, Symbalta, and aspirin. No changes! Anyone have any good solution? […] I seemed to have gotten a good deal of relief with Plaquenil (Have SLE and Sjogren’s) but had to discontinue due to side effects. Since stopping the Plaquenil symptoms have begun to return. Many have success with that medication, but some of us don’t tolerate it well.

• #19 Misoprostol Effective in Management of Erythromelalgialogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/jd200405250000005/2004/05/25/misoprostol-effective-management-erythromelalgia

  Erythromelalgia is a difficult disease to treat. Iloprost, a prostacyclin analog, was shown to be effective by an investigative team; the investigators now report control of erythromelalgia symptoms with oral misoprostol. This agent is indicated for prevention of gastrointestinal ulceration from nonsteroidal anti-inflammatory drugs, constipation, early pregnancy termination, and cervical ripening at delivery. […] Current therapies for erythromelalgia are usually ineffective, unless an underlying problem is identified and treated. These results suggest that misoprostol — an inexpensive, readily available therapy — may be useful. This drug should not be given to pregnant women and should be used cautiously in women who have had a cesarean section or major uterine surgery and in those who are breast-feeding.

• #20 Secondary erythromelalgia successfully treated with patient‑controlled epidural analgesia and interferon α‑2b: A case report and review of the literature

  https://www.spandidos-publications.com/10.3892/etm.2016.3088

  Erythromelalgia (EM) is a debilitating neurovascular disease that is refractory to numerous treatment modalities. […] The study aimed to provide clinical information on this condition based on the findings of the present case and previously reported cases. PCEA containing local anesthetics and dexamethasone or fentanyl resulted in nearly complete relief of symptoms. Furthermore, continuous use of interferon 2b for the treatment of the PV completely relieved the EM symptoms. In conclusion, PCEA and interferon 2b may be promising treatments for EM secondary to PV. […] Interferon therapy, which inhibits PLT generation, was more effective than aspirin, which inhibits PLT aggregation. PCEA acts on certain pathophysiological aspects of EM, while interferon targets the protopathy; however, PCEA is able to produce long-term effects after one treatment cycle, while interferon requires persistent use and may result in certain side effects. Identification of the optimal modality for the treatment of EM secondary to PV will require further studies.

• #21 Sympathetic Block for Treating Primary Erythromelalgia

  https://www.epain.org/journal/view.html?doi=10.3344/kjp.2010.23.1.55

  Primary erythromelalgia is a rare condition that’s characterized by erythema, an increased skin temperature and burning pain in the extremities. […] Our experience with this disease demonstrates that sympathetic blocks are effective in improving the symptoms and they may be attempted on erythromelalgia patients who do not respond to other treatments, including medication and epidural blocks. […] There are many types of treatment for erythromelalgia. There are oral treatments such as neuroleptic drugs and vasodilator. Some kind of drug can be administered by intravenous injection. Sympathetic ganglion block and epidural block are effective invasive procedures to treat this condition. […] Because the disease affects the patient’s quality of life, 50% of the patients cannot take long walks, 12.5% have to leave work, 3% have to use a wheelchair and 2.1% are bed-ridden.

• #22 Case Report and Literature Review: Interventional Management of Erythromelalgia

  http://www.transpopmed.org/articles/tppm/tppm-2019-6-094.php

  Erythromelalgia is a rare and very difficult to treat pain syndrome that usually presents as severe bilateral burning pain in the extremities. […] We suggest epidural infusion as the first line interventional management, followed by sympathetic block. Spinal cord stimulation can be considered if other interventional managements fail. […] Medical treatment is the first line of therapies for the erythromelalgia. […] An epidural infusion should be considered second line as the risks are low and relief can be significant and lasting in a variety of cases. […] If epidural infusion does not offer good relief, or if it is not lasting, sympathetic blockade should be considered. […] With a good response, neurolysis can be achieved and may offer long-lasting relief. […] Finally, with certain cases, especially those with contraindications to spinal cord stimulator, thalamic electrode implantation may be considered.

• #23 Sympathetic Block for Treating Primary Erythromelalgia

  https://www.epain.org/journal/view.html?doi=10.3344/kjp.2010.23.1.55

  So doctor must actively try to alleviate the patient’s pain with various treatments in order for the patients to continue their normal life-activities. […] If symptoms are not alleviated with oral medication, then venous administration of nitroprusside, lidocaine and prostaglandin has been reported to mitigate these patients’ symptoms. […] The invasive procedures for erythromelalgia include epidural block, sympathetic ganglion block, spinal cord stimulator insertion and brachial plexus block. […] A sympathetic block may be an effective for those cases that are refractory to non-invasive treatment.

• #24 Erythromelalgia: Causes, Symptoms, and Treatment

  https://www.medicoverhospitals.in/diseases/erythromelalgia/

  Preventing erythromelalgia can be challenging, especially in cases with a genetic basis. However, some measures can help manage and reduce the frequency of episodes: […] Regular Monitoring: Regular check-ups with a healthcare provider to monitor the condition and adjust treatment as needed. […] Healthy Lifestyle: Maintaining a healthy lifestyle, including a balanced diet and regular exercise, while avoiding known triggers. […] Stress Management: Techniques like mindfulness, meditation, and counseling to manage stress effectively. […] Prevention includes avoiding heat exposure, staying cool, and managing any underlying health conditions that might trigger symptoms.

• #25 Erythromelalgia – MD Searchlight

  https://mdsearchlight.com/blood-disorders/erythromelalgia/

  People suffering from the two types of a condition called erythromelalgia, whether its primary (meaning it occurs on its own) or secondary (meaning its caused by another disease), need to have a clear understanding of the causes of the disease. A comprehensive check-up is needed to rule out certain diseases of the blood cells (myeloproliferative disorders) which are known to cause secondary erythromelalgia. This is an important step in both managing erythromelalgia and quickly diagnosing any underlying conditions. […] Regular check-ups are necessary to find out how effective the treatment is. Its also recommended that patients have a complete blood count with differential done yearly. This is a type of blood test which counts the different types of cells in your blood. This test helps doctors keep an eye on the development of any myeloproliferative disorder.

• #26

  https://link.springer.com/article/10.1007/s11936-006-0008-8

  No treatment is consistently effective in the management of patients with erythromelalgia. […] The management of erythromelalgia is difficult and frequently involves a multidisciplinary approach. An approach to management of individuals with erythromelalgia includes patient education, learning to avoid episodes, relieving discomfort of the episodes, controlling secondary and underlying factors, and use of drugs used to control erythromelalgia.

• #27 What Is Erythromelalgia?

  https://www.webmd.com/heart-disease/what-is-erythromelalgia

  Its also important to take care of your mental health when dealing with erythromelalgia. Because its a rare disorder, its hard for most people to understand what youre going through. You may end up feeling isolated as a result. To help you not feel so alone, you can try to reach out to online erythromelalgia support groups to share your story and hear the stories of others.

• #28 A large temperature fluctuation may trigger an epidemic erythromelalgia outbreak in China | Scientific Reports

  https://www.nature.com/articles/srep09525

  Epidemic EM appears quite common in southern China. […] The V-shaped fluctuation of daily average temperature during the late winter and early spring might trigger an epidemic EM outbreak. […] We suggest that epidemic EM should be included in the current routine disease surveillance system and establish an early warning system of epidemic EM based on measured or projected temperature fluctuation in China.

• #29 Erythromelalgia – UpToDate

  https://www.uptodate.com/contents/erythromelalgia

  Erythromelalgia is a rare, acquired or (very rarely) inherited clinical syndrome of intermittently red, hot, painful extremities. Patients instinctively try to relieve symptoms by cooling the involved areas with fans, cold water, or ice. […] There is no cure for erythromelalgia; therefore, treatment is focused on improving symptoms. Many patients can be successfully managed with behavioral interventions, topical medications, and aspirin. Other interventions may be beneficial when these measures are insufficient. […] The epidemiology, clinical features, diagnosis, and management of erythromelalgia will be reviewed here.

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