Materiały źródłowe

• #1 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  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.

• #2 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Erythromelalgia (EM) is a rare disorder characterized by erythematous, warm, painful extremities, which is often precipitated by cold conditions. […] The management of pain in EM is challenging as no single therapy has been found to be effective. […] There is currently no consensus or guidelines on management of pain in EM. […] To date, there is no therapy that has demonstrated consistently high efficacy across individuals with EM, highlighted by the lack of consensus or guidelines on the treatment of EM. […] The aim of this study was to conduct a systematic review of pain treatment for EM and provide an update on treatment options. […] Patients with EM often employ strategies to avoid situations that may precipitate pain (typically induced by heat) and the use of cold exposure reduction.

• #3 Erythromelalgia – UpToDate

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

  Erythromelalgia is a rare, acquired or (very rarely) inherited clinical syndrome of intermittently red, hot, painful extremities. […] 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.

• #4 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia treatment often includes drugs you take by mouth. Many of these drugs are for other medical conditions. But they can be effective in relieving erythromelalgia symptoms. […] The treatment thats right for you depends on the cause. For secondary erythromelalgia, treating the underlying issue may relieve your symptoms. Treatment for primary erythromelalgia includes medications. […] Erythromelalgia treatment may also include: Lidocaine infusions, a pain medication you receive through a vein in your arm. Nutritional supplements, such as magnesium and alpha-lipoic acid. Pain management therapies, including nerve blocks. Topical medications, such as creams you apply to your skin. […] Nonmedical therapies include: Elevating the affected area. Resting on a cool surface, such as a tile floor. Using a fan. Wrapping cool first-aid gel packs in a thin towel and placing them on your skin.

• #5 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia is a rare condition that causes episodes of pain and burning skin. There are many treatment options, and they typically include medications. Sometimes medications arent enough to quiet symptom flares, making it difficult to go about your daily routine. Even with these setbacks, many people live long, rewarding lives.

• #6 Erythromelalgia: A Very Rare Condition

  https://www.verywellhealth.com/erythromelalgia-overview-4582735

  Erythromelalgia Treatment, therapy […] Treatment options should follow a stepwise approach that begins with nonpharmacological measures, followed by topical medicines, oral medications, pain rehabilitation programs, and, in extreme cases, surgery. It may take some time to find a treatment plan that works. […] There is no single treatment for EM, and there is also no cure. In secondary EM, treating the underlying condition may bring about relief. Most of the time, however, EM cannot be fully treated. In these situations, symptom relief is the goal. […] Common treatments used to control EM symptoms: Aspirin, Carbamazepine, Gabapentin, Sodium nitroprusside, Nitroglycerin, Antihistamines, Sodium ion channel blockers, Prostaglandins. […] These medications can be used in combination as needed. Other less commonly used treatments have provided relief, such as itraconazole, an antifungal. […] Cooling the affected areas with cold packs and elevating flaring feet can help with coping. […] Therapies such as biofeedback and cognitive behavioral therapy may work for some people. […] Nerve blocks are among the treatments that are sometimes used. In some cases, repeat nerve blocks may be needed, but the condition can resolve after just one treatment. […] Surgery is considered when no other treatments provide relief. A surgical procedure, called a sympathectomy, cuts nerve endings that transmit pain signals to the hands and feet. There are serious risks associated with this surgery, including bleeding, infection, and nerve damage. Doctors will consider sympathectomy only when a person’s quality of life is significantly affected by EM. […] Though there is no cure for EM, the outlook can still be positive. Treatment may bring about complete relief of symptoms. Most of the time, however, EM cannot be fully treated. Treatment takes some trial and error to find a plan that works to relieve symptoms.

• #7 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Various classes of antidepressants have been used. […] Gabapentinoids such as gabapentin and pregabalin are commonly used to treat neuropathic pain. […] Antihistamines may have potent vascular effects and may be considered for treatment. […] Procedural interventions have been reported, and range from less-invasive techniques such as injection of botulinum toxin and sympathetic blockade to highly invasive procedures such as implantation of spinal cord stimulators. […] The efficacy of an intensive pain rehabilitation program has been evaluated in eight adults with EM. […] There has been substantial advancement in the understanding of the pathophysiology underlying EM. […] Most patients with EM require multimodal pharmacotherapy, specifically targeting both pathophysiology and neuropathic pain. […] A common starting point would be to trial agents with least risk and most likely efficacy. […] Finally, physical and occupational therapy, rehabilitation, and pain psychology treatment would also be recommended.

• #8 Current pain management strategies for patients with erythromelalgia: | JPR

  https://www.dovepress.com/current-pain-management-strategies-for-patients-with-erythromelalgia-a-peer-reviewed-fulltext-article-JPR

  Many pharmacologic agents have been used to treat EM over the decades. […] Aspirin has been considered as a first-line treatment, likely due to early studies reporting its efficacy and the low side effect profile. […] There appears to be a limited role for nonsteroidal anti-inflammatory drugs other than aspirin in the treatment of EM. […] Sodium channel blockers have proved promising in the preclinical setting. […] Positive response to lidocaine infusions has been considered as an indicator of success with mexiletine, an orally available form of lidocaine. […] Most patients with EM require multimodal pharmacotherapy, specifically targeting both pathophysiology and neuropathic pain. […] A common starting point would be to trial agents with least risk and most likely efficacy. […] Finally, physical and occupational therapy, rehabilitation, and pain psychology treatment would also be recommended.

• #9 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  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.

• #10 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  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.

• #11 Erythromelalgia – Wikipedia

  https://en.wikipedia.org/wiki/Erythromelalgia

  Specific management tactics include avoidance of attack triggers such as: heat, change in temperature, exercise or over exertion, alcohol and spicy foods. […] One clinical study has demonstrated the efficacy of IV lidocaine or oral mexilitine, though differences between the primary and secondary forms were not studied. […] Strong anecdotal evidence from EM patients shows that a combination of drugs such as duloxetine and pregabalin is an effective way of reducing the stabbing pains and burning sensation symptoms of erythromelalgia in conjunction with the appropriate analgesia. […] In some cases, antihistamines may give some relief. […] Some suffering with EM are prescribed ketamine topical creams as a way of managing pain on a long-term basis. […] Living with erythromelalgia can result in a deterioration in quality of life resulting in the inability to function in a work place, lack of mobility, depression, and is socially alienating; much greater education of medical practitioners is needed.

• #12 What Is Erythromelalgia?

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

  Magnesium, alpha-lipoic acid, and other nutritional supplements […] Pain management therapies […] Tropical medications in the form of creams. […] There are also some nonmedical treatment options available that you can practice at home. These include: Elevating the affected area […] Allowing the affected area to rest in a cool area […] Using a fan to cool yourself down […] Wrapping first-aid gel packs in a towel and applying them to your affected skin. […] Some patients may want to soak painful and red areas in ice water or to press an icy compress against the affected area. This can, however, cause erythromelalgia symptoms to worsen. Additionally, exposing your skin to icy conditions may put you at risk for certain skin issues such as nonhealing wounds and necrosis.

• #13 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia treatment often includes drugs you take by mouth. Many of these drugs are for other medical conditions. But they can be effective in relieving erythromelalgia symptoms. […] The treatment thats right for you depends on the cause. For secondary erythromelalgia, treating the underlying issue may relieve your symptoms. Treatment for primary erythromelalgia includes medications. […] Erythromelalgia treatment may also include: Lidocaine infusions, a pain medication you receive through a vein in your arm. Nutritional supplements, such as magnesium and alpha-lipoic acid. Pain management therapies, including nerve blocks. Topical medications, such as creams you apply to your skin. […] Nonmedical therapies include: Elevating the affected area. Resting on a cool surface, such as a tile floor. Using a fan. Wrapping cool first-aid gel packs in a thin towel and placing them on your skin.

• #14 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Evaluation and treatment can be conducted in an outpatient setting. Treatment is primarily medical and supportive. Local measures, such as cooling or elevating the extremity, may relieve symptoms. Avoid excessive warming or dependency of the extremity. The environment should be modified so that it is not too hot. […] A number of topical and systemic medications have been used for treatment of erythromelalgia. As current therapies provide only symptomatic relief, topical medications are generally preferred, in view of their more limited adverse effects. […] Case reports describe successful use of spinal cord stimulation and ganglion stimulation and blocks. […] With secondary erythromelalgia, treatment of the underlying disease process may lead to improvement or resolution of the erythromelalgia.

• #15 Erythromelalgia – Heart and Blood Vessel Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/heart-and-blood-vessel-disorders/peripheral-arterial-disease/erythromelalgia

  Erythromelalgia treatment includes avoiding exposure to heat, resting, elevating the legs or arms, and applying cold packs to the legs or arms or immersing them in cold water. These measures sometimes relieve symptoms or prevent attacks. […] If an underlying disease that causes erythromelalgia is identified, treating that disease may relieve symptoms. If no underlying disorder is identified, gabapentin may relieve symptoms. Aspirin may be helpful when a myeloproliferative disorder is the present.

• #16 Erythromelalgia

  https://dermnetnz.org/topics/erythromelalgia

  What treatment is available for erythromelalgia? The underlying cause must be treated where possible in secondary erythromelalgia. The treatment of symptoms of both primary and secondary erythromelalgia is through general non-medical measures, drug therapy and surgical intervention. Most cases of primary erythromelalgia are refractory to pharmacotherapy and response to pain therapeutics is variable. Most of these therapies have only been validated in case reports or smaller studies. […] Cooling or elevating the extremity may relieve symptoms. Care is needed around cold water immersions, although it will provide temporary relief, it can cause many other serious problems. Frequent immersion into cold water can create a vicious cycle, as the changes in temperature may cause reactive flaring. This can also lead to maceration of the skin, non-healing ulcers, infection, gangrene and amputation.

• #17 What Is Erythromelalgia?

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

  Magnesium, alpha-lipoic acid, and other nutritional supplements […] Pain management therapies […] Tropical medications in the form of creams. […] There are also some nonmedical treatment options available that you can practice at home. These include: Elevating the affected area […] Allowing the affected area to rest in a cool area […] Using a fan to cool yourself down […] Wrapping first-aid gel packs in a towel and applying them to your affected skin. […] Some patients may want to soak painful and red areas in ice water or to press an icy compress against the affected area. This can, however, cause erythromelalgia symptoms to worsen. Additionally, exposing your skin to icy conditions may put you at risk for certain skin issues such as nonhealing wounds and necrosis.

• #18 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Evaluation and treatment can be conducted in an outpatient setting. Treatment is primarily medical and supportive. Local measures, such as cooling or elevating the extremity, may relieve symptoms. Avoid excessive warming or dependency of the extremity. The environment should be modified so that it is not too hot. […] A number of topical and systemic medications have been used for treatment of erythromelalgia. As current therapies provide only symptomatic relief, topical medications are generally preferred, in view of their more limited adverse effects. […] Case reports describe successful use of spinal cord stimulation and ganglion stimulation and blocks. […] With secondary erythromelalgia, treatment of the underlying disease process may lead to improvement or resolution of the erythromelalgia.

• #19 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Some studies have shown that topical lidocaine patches, compounded topical amitriptyline-ketamine, and topical capsaicin applied three times daily may improve the pain associated with erythromelalgia. While 0.2% midodrine compounded in a moisturizing cream applied thrice a day might improve redness associated with erythromelalgia. Topical therapy should be continued for two to four weeks to assess the efficacy. […] Aspirin is the drug of choice for erythromelalgia associated with thrombocytopenia or myeloproliferative disorder. Other NSAIDs like anagrelide may be used as an alternative. Diagnosing and treating the underlying myeloproliferative disorder is of utmost importance for the improvement of erythromelalgia. In this patient population, the addition of hydroxyurea (chemotherapy) to reduce the platelet count may also improve the symptoms of erythromelalgia, while phlebotomy might be useful for patients with polycythemia vera.

• #20 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, nitroglycerin, gabapentin, typhoid vaccine, and topical oxymetazoline appear in the literature, as well as case series with intravenous bupivacaine, and lidocaine plus mexiletine. […] Medications that affect voltage-gated sodium channels (eg, lidocaine and its oral form, mexiletine) show promise. […] Prostacyclin may provide some benefit. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analogue) improved symptoms. […] A double-blind, crossover, placebo-compared study of the prostaglandin E1 analog misoprostol in 21 patients with erythromelalgia found that it reduces symptoms and microvascular arteriovenous shunting. […] Topical therapy has been studied, including transdermal lidocaine.

• #21 Erythromelalgia

  https://dermnetnz.org/topics/erythromelalgia

  Topical capsaicin cream has been reported with varying results. […] Aspirin relieves symptoms of erythromelalgia related to thrombocytosis. Sodium channel blockers mexiletine has been shown to improve pain, especially in patients with V872G mutation. Calcium antagonists nifedipine, diltiazem. Prostaglandins misoprostol. Intravenous infusions of nitroprusside, lignocaine (lidocaine) and prostaglandin. Medications used for neuropathic pain include serotonin re-uptake inhibitors venlafaxine, sertraline, fluoxetine, paroxetine, tricyclic antidepressants amitriptyline, imipramine, and anticonvulsants gabapentin, carbamazepine. […] Several drugs are undergoing clinical trials in primary erythromelalgia. Examples include Nav1.7 channel modulators (PF-05089771) and NAV1.7 channel blockers (TV-45070). […] Surgical sympathectomy (a procedure in which the sympathetic nerve fibres are selectively cut) includes thoracic sympathectomy for hands and lumbar sympathectomy for feet. Patients respond quite variably and no single therapy has proved consistently effective.

• #22 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  A study in 12 patients reported benefit with the alpha1-agonist midodrine, 0.2%, compounded in a moisturizing skin cream and applied topically three times a day during symptoms. […] Poterucha et al treated 36 patients with compounded topical amitriptyline-ketamine; 75% of those patients reported a reduction in pain. […] A Mayo Clinic study in 31 patients found that response to treatment with corticosteroids was more likely to occur in patients who reported a precipitant for their erythromelalgia (eg, surgery, trauma, or infection) and in those who had a subacute temporal profile to disease zenith (21 days). […] In cases that are associated with other disorders, treating the original disease might improve symptoms. […] For erythromelalgia related to thrombocytosis, aspirin is usually the treatment of choice. Other nonsteroidal anti-inflammatory drugs (NSAIDs) provide relief of short duration. Anagrelide may be an alternative. Other platelet-inhibiting agents (eg, ticlopidine and dipyridamole) have no effect. […] Monitor for complications, response to treatment, and development of a myeloproliferative disorder because erythromelalgia often precedes the clinical appearance of polycythemia vera or essential thrombocythemia.

• #23 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  A study in 12 patients reported benefit with the alpha1-agonist midodrine, 0.2%, compounded in a moisturizing skin cream and applied topically three times a day during symptoms. […] Poterucha et al treated 36 patients with compounded topical amitriptyline-ketamine; 75% of those patients reported a reduction in pain. […] A Mayo Clinic study in 31 patients found that response to treatment with corticosteroids was more likely to occur in patients who reported a precipitant for their erythromelalgia (eg, surgery, trauma, or infection) and in those who had a subacute temporal profile to disease zenith (21 days). […] In cases that are associated with other disorders, treating the original disease might improve symptoms. […] For erythromelalgia related to thrombocytosis, aspirin is usually the treatment of choice. Other nonsteroidal anti-inflammatory drugs (NSAIDs) provide relief of short duration. Anagrelide may be an alternative. Other platelet-inhibiting agents (eg, ticlopidine and dipyridamole) have no effect. […] Monitor for complications, response to treatment, and development of a myeloproliferative disorder because erythromelalgia often precedes the clinical appearance of polycythemia vera or essential thrombocythemia.

• #24 Erythromelalgia – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/erythromelalgia/

  Medical treatments for erythromelalgia: There are several different medications that can be used to try and relieve symptoms. Sometimes people may need to try several different medications or combinations of medications to find what helps them. Treatments that your doctor may use include: Capsaicin cream or doxepin cream on the affected area. Medications which may help reduce pain caused by increased sensitivity of the nervous system: Anti-convulsant medications: e.g. Gabapentin, Pregabalin, carbamazepine. Newer antidepressants: e.g. venlafaxine, sertraline, fluoxetine, paroxetine. Tricyclic antidepressants: e.g. amitriptyline. Calcium channel blockers (e.g. nifedipine, diltiazem) which can alter blood flow. Intravenous infusions at the hospital (medicine through a drip) of nitroprusside, prostaglandin (iloprost) or lignocaine (lidocaine). Mexilitine tablets work similarly to lignocaine, but can be difficult for hospitals to obtain. A lidocaine patch applied to the skin may also help. Nerve blocks or surgical sympathectomy (a procedure in which sympathetic nerve fibres are cut) have helped some people, but not others. Sympathectomy can cause permanent severe side effects in some people. […] If you experience any psychological distress associated with the condition, you should talk about this with your doctor. Cognitive behavioural therapy and other talking therapies can help some people cope with the distress the condition may cause.

• #25 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Some studies have shown that topical lidocaine patches, compounded topical amitriptyline-ketamine, and topical capsaicin applied three times daily may improve the pain associated with erythromelalgia. While 0.2% midodrine compounded in a moisturizing cream applied thrice a day might improve redness associated with erythromelalgia. Topical therapy should be continued for two to four weeks to assess the efficacy. […] Aspirin is the drug of choice for erythromelalgia associated with thrombocytopenia or myeloproliferative disorder. Other NSAIDs like anagrelide may be used as an alternative. Diagnosing and treating the underlying myeloproliferative disorder is of utmost importance for the improvement of erythromelalgia. In this patient population, the addition of hydroxyurea (chemotherapy) to reduce the platelet count may also improve the symptoms of erythromelalgia, while phlebotomy might be useful for patients with polycythemia vera.

• #26 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Many pharmacologic agents have been used to treat EM over the decades. […] Aspirin has been considered as a first-line treatment, likely due to early studies reporting its efficacy and the low side effect profile. […] There appears to be a limited role for nonsteroidal anti-inflammatory drugs other than aspirin in the treatment of EM. […] Prostaglandin and prostacyclin analogs with vasodilatory effects have been the subjects of two small RCTs in EM treatment of pain. […] Drugs that target the underlying genetic cause of cases of hereditary EM (Nav1.7 sodium channel) have proved promising in the preclinical setting. […] Lidocaine and mexiletine provide nonselective partial blockade of voltage-gated sodium channels, working on segment 6 of domain IV of the alpha subunit to shorten the action potential duration.

• #27 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  In patients with myeloproliferative disorders, chemotherapy to reduce the platelet count often alleviates symptoms, but it is not universally effective. Some patients with polycythemia vera have responded to phlebotomy. A hematologist may be consulted if an associated myeloproliferative disorder is present. […] Surgery probably has no role in the management of erythromelalgia except to treat the rare complication of gangrene. Surgical sympathectomy has been attempted, with variable results. […] For primary erythromelalgia, the best therapy is unknown. A wide range of agents has been studied, including the following: Vascular agents – Aspirin, prostaglandins; Sodium channel blockers – Lidocaine, mexiletine, carbamazepine, oxcarbazepine; Calcium channel blockers – Amlodipine, nifedipine, diltiazem, high-dose oral magnesium; Antidepressants – Tricyclic antidepressants, serotonin reuptake inhibitors; Anticonvulsants – Gabapentin, pregabalin; Antihistamines; Immunosuppressants.

• #28 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Some studies have shown that topical lidocaine patches, compounded topical amitriptyline-ketamine, and topical capsaicin applied three times daily may improve the pain associated with erythromelalgia. While 0.2% midodrine compounded in a moisturizing cream applied thrice a day might improve redness associated with erythromelalgia. Topical therapy should be continued for two to four weeks to assess the efficacy. […] Aspirin is the drug of choice for erythromelalgia associated with thrombocytopenia or myeloproliferative disorder. Other NSAIDs like anagrelide may be used as an alternative. Diagnosing and treating the underlying myeloproliferative disorder is of utmost importance for the improvement of erythromelalgia. In this patient population, the addition of hydroxyurea (chemotherapy) to reduce the platelet count may also improve the symptoms of erythromelalgia, while phlebotomy might be useful for patients with polycythemia vera.

• #29 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Primary erythromelalgia can be particularly resistant to treatment. Medications affecting the voltage-gated sodium channels (lidocaine, mexiletine, and carbamazepine) have shown promise in primary erythromelalgia. There is some evidence for the use of mexiletine 100 mg to 200 mg three times a day. An alternative regimen consists of carbamazepine 300 mg twice per day with gabapentin, which is titrated up to 300 mg five times a day. Secondary erythromelalgia may be responsive to aspirin, 81 mg to 640 mg daily, and is often used as a first-line agent. […] Other agents that may be effective in treating primary and secondary erythromelalgia include gabapentin, pregabalin, venlafaxine, amitriptyline, iloprost, and misoprostol, calcium channel blockers, and beta-blockers. However, these agents are less well studied, and no specific treatment regimen has been proposed.

• #30 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Primary erythromelalgia can be particularly resistant to treatment. Medications affecting the voltage-gated sodium channels (lidocaine, mexiletine, and carbamazepine) have shown promise in primary erythromelalgia. There is some evidence for the use of mexiletine 100 mg to 200 mg three times a day. An alternative regimen consists of carbamazepine 300 mg twice per day with gabapentin, which is titrated up to 300 mg five times a day. Secondary erythromelalgia may be responsive to aspirin, 81 mg to 640 mg daily, and is often used as a first-line agent. […] Other agents that may be effective in treating primary and secondary erythromelalgia include gabapentin, pregabalin, venlafaxine, amitriptyline, iloprost, and misoprostol, calcium channel blockers, and beta-blockers. However, these agents are less well studied, and no specific treatment regimen has been proposed.

• #31 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Primary erythromelalgia can be particularly resistant to treatment. Medications affecting the voltage-gated sodium channels (lidocaine, mexiletine, and carbamazepine) have shown promise in primary erythromelalgia. There is some evidence for the use of mexiletine 100 mg to 200 mg three times a day. An alternative regimen consists of carbamazepine 300 mg twice per day with gabapentin, which is titrated up to 300 mg five times a day. Secondary erythromelalgia may be responsive to aspirin, 81 mg to 640 mg daily, and is often used as a first-line agent. […] Other agents that may be effective in treating primary and secondary erythromelalgia include gabapentin, pregabalin, venlafaxine, amitriptyline, iloprost, and misoprostol, calcium channel blockers, and beta-blockers. However, these agents are less well studied, and no specific treatment regimen has been proposed.

• #32 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  In patients with myeloproliferative disorders, chemotherapy to reduce the platelet count often alleviates symptoms, but it is not universally effective. Some patients with polycythemia vera have responded to phlebotomy. A hematologist may be consulted if an associated myeloproliferative disorder is present. […] Surgery probably has no role in the management of erythromelalgia except to treat the rare complication of gangrene. Surgical sympathectomy has been attempted, with variable results. […] For primary erythromelalgia, the best therapy is unknown. A wide range of agents has been studied, including the following: Vascular agents – Aspirin, prostaglandins; Sodium channel blockers – Lidocaine, mexiletine, carbamazepine, oxcarbazepine; Calcium channel blockers – Amlodipine, nifedipine, diltiazem, high-dose oral magnesium; Antidepressants – Tricyclic antidepressants, serotonin reuptake inhibitors; Anticonvulsants – Gabapentin, pregabalin; Antihistamines; Immunosuppressants.

• #33 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Primary erythromelalgia can be particularly resistant to treatment. Medications affecting the voltage-gated sodium channels (lidocaine, mexiletine, and carbamazepine) have shown promise in primary erythromelalgia. There is some evidence for the use of mexiletine 100 mg to 200 mg three times a day. An alternative regimen consists of carbamazepine 300 mg twice per day with gabapentin, which is titrated up to 300 mg five times a day. Secondary erythromelalgia may be responsive to aspirin, 81 mg to 640 mg daily, and is often used as a first-line agent. […] Other agents that may be effective in treating primary and secondary erythromelalgia include gabapentin, pregabalin, venlafaxine, amitriptyline, iloprost, and misoprostol, calcium channel blockers, and beta-blockers. However, these agents are less well studied, and no specific treatment regimen has been proposed.

• #34 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Various classes of antidepressants have been used. […] Gabapentinoids such as gabapentin and pregabalin are commonly used to treat neuropathic pain. […] Antihistamines may have potent vascular effects and may be considered for treatment. […] Procedural interventions have been reported, and range from less-invasive techniques such as injection of botulinum toxin and sympathetic blockade to highly invasive procedures such as implantation of spinal cord stimulators. […] The efficacy of an intensive pain rehabilitation program has been evaluated in eight adults with EM. […] There has been substantial advancement in the understanding of the pathophysiology underlying EM. […] Most patients with EM require multimodal pharmacotherapy, specifically targeting both pathophysiology and neuropathic pain. […] A common starting point would be to trial agents with least risk and most likely efficacy. […] Finally, physical and occupational therapy, rehabilitation, and pain psychology treatment would also be recommended.

• #35 Erythromelalgia

  https://dermnetnz.org/topics/erythromelalgia

  Topical capsaicin cream has been reported with varying results. […] Aspirin relieves symptoms of erythromelalgia related to thrombocytosis. Sodium channel blockers mexiletine has been shown to improve pain, especially in patients with V872G mutation. Calcium antagonists nifedipine, diltiazem. Prostaglandins misoprostol. Intravenous infusions of nitroprusside, lignocaine (lidocaine) and prostaglandin. Medications used for neuropathic pain include serotonin re-uptake inhibitors venlafaxine, sertraline, fluoxetine, paroxetine, tricyclic antidepressants amitriptyline, imipramine, and anticonvulsants gabapentin, carbamazepine. […] Several drugs are undergoing clinical trials in primary erythromelalgia. Examples include Nav1.7 channel modulators (PF-05089771) and NAV1.7 channel blockers (TV-45070). […] Surgical sympathectomy (a procedure in which the sympathetic nerve fibres are selectively cut) includes thoracic sympathectomy for hands and lumbar sympathectomy for feet. Patients respond quite variably and no single therapy has proved consistently effective.

• #36 Erythromelalgia

  https://dermnetnz.org/topics/erythromelalgia

  Topical capsaicin cream has been reported with varying results. […] Aspirin relieves symptoms of erythromelalgia related to thrombocytosis. Sodium channel blockers mexiletine has been shown to improve pain, especially in patients with V872G mutation. Calcium antagonists nifedipine, diltiazem. Prostaglandins misoprostol. Intravenous infusions of nitroprusside, lignocaine (lidocaine) and prostaglandin. Medications used for neuropathic pain include serotonin re-uptake inhibitors venlafaxine, sertraline, fluoxetine, paroxetine, tricyclic antidepressants amitriptyline, imipramine, and anticonvulsants gabapentin, carbamazepine. […] Several drugs are undergoing clinical trials in primary erythromelalgia. Examples include Nav1.7 channel modulators (PF-05089771) and NAV1.7 channel blockers (TV-45070). […] Surgical sympathectomy (a procedure in which the sympathetic nerve fibres are selectively cut) includes thoracic sympathectomy for hands and lumbar sympathectomy for feet. Patients respond quite variably and no single therapy has proved consistently effective.

• #37 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Primary erythromelalgia can be particularly resistant to treatment. Medications affecting the voltage-gated sodium channels (lidocaine, mexiletine, and carbamazepine) have shown promise in primary erythromelalgia. There is some evidence for the use of mexiletine 100 mg to 200 mg three times a day. An alternative regimen consists of carbamazepine 300 mg twice per day with gabapentin, which is titrated up to 300 mg five times a day. Secondary erythromelalgia may be responsive to aspirin, 81 mg to 640 mg daily, and is often used as a first-line agent. […] Other agents that may be effective in treating primary and secondary erythromelalgia include gabapentin, pregabalin, venlafaxine, amitriptyline, iloprost, and misoprostol, calcium channel blockers, and beta-blockers. However, these agents are less well studied, and no specific treatment regimen has been proposed.

• #38 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  In patients with myeloproliferative disorders, chemotherapy to reduce the platelet count often alleviates symptoms, but it is not universally effective. Some patients with polycythemia vera have responded to phlebotomy. A hematologist may be consulted if an associated myeloproliferative disorder is present. […] Surgery probably has no role in the management of erythromelalgia except to treat the rare complication of gangrene. Surgical sympathectomy has been attempted, with variable results. […] For primary erythromelalgia, the best therapy is unknown. A wide range of agents has been studied, including the following: Vascular agents – Aspirin, prostaglandins; Sodium channel blockers – Lidocaine, mexiletine, carbamazepine, oxcarbazepine; Calcium channel blockers – Amlodipine, nifedipine, diltiazem, high-dose oral magnesium; Antidepressants – Tricyclic antidepressants, serotonin reuptake inhibitors; Anticonvulsants – Gabapentin, pregabalin; Antihistamines; Immunosuppressants.

• #39 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, nitroglycerin, gabapentin, typhoid vaccine, and topical oxymetazoline appear in the literature, as well as case series with intravenous bupivacaine, and lidocaine plus mexiletine. […] Medications that affect voltage-gated sodium channels (eg, lidocaine and its oral form, mexiletine) show promise. […] Prostacyclin may provide some benefit. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analogue) improved symptoms. […] A double-blind, crossover, placebo-compared study of the prostaglandin E1 analog misoprostol in 21 patients with erythromelalgia found that it reduces symptoms and microvascular arteriovenous shunting. […] Topical therapy has been studied, including transdermal lidocaine.

• #40 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, nitroglycerin, gabapentin, typhoid vaccine, and topical oxymetazoline appear in the literature, as well as case series with intravenous bupivacaine, and lidocaine plus mexiletine. […] Medications that affect voltage-gated sodium channels (eg, lidocaine and its oral form, mexiletine) show promise. […] Prostacyclin may provide some benefit. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analogue) improved symptoms. […] A double-blind, crossover, placebo-compared study of the prostaglandin E1 analog misoprostol in 21 patients with erythromelalgia found that it reduces symptoms and microvascular arteriovenous shunting. […] Topical therapy has been studied, including transdermal lidocaine.

• #41 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Various classes of antidepressants have been used. […] Gabapentinoids such as gabapentin and pregabalin are commonly used to treat neuropathic pain. […] Antihistamines may have potent vascular effects and may be considered for treatment. […] Procedural interventions have been reported, and range from less-invasive techniques such as injection of botulinum toxin and sympathetic blockade to highly invasive procedures such as implantation of spinal cord stimulators. […] The efficacy of an intensive pain rehabilitation program has been evaluated in eight adults with EM. […] There has been substantial advancement in the understanding of the pathophysiology underlying EM. […] Most patients with EM require multimodal pharmacotherapy, specifically targeting both pathophysiology and neuropathic pain. […] A common starting point would be to trial agents with least risk and most likely efficacy. […] Finally, physical and occupational therapy, rehabilitation, and pain psychology treatment would also be recommended.

• #42 Erythromelalgia: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/200071-overview

  High-dose oral magnesium treatment of chronic, intractable erythromelalgia. […] Erythromelalgia successfully treated with low-dose gabapentin in a pediatric patient. […] Successful treatment of knee erythromelalgia with topical oxymetazoline. […] Experience with oral mexiletine in primary erythromelalgia in children.

• #43 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia treatment often includes drugs you take by mouth. Many of these drugs are for other medical conditions. But they can be effective in relieving erythromelalgia symptoms. […] The treatment thats right for you depends on the cause. For secondary erythromelalgia, treating the underlying issue may relieve your symptoms. Treatment for primary erythromelalgia includes medications. […] Erythromelalgia treatment may also include: Lidocaine infusions, a pain medication you receive through a vein in your arm. Nutritional supplements, such as magnesium and alpha-lipoic acid. Pain management therapies, including nerve blocks. Topical medications, such as creams you apply to your skin. […] Nonmedical therapies include: Elevating the affected area. Resting on a cool surface, such as a tile floor. Using a fan. Wrapping cool first-aid gel packs in a thin towel and placing them on your skin.

• #44 Erythromelalgia: Symptoms, Causes, Treatment & More

  https://www.healthline.com/health/erythromelalgia

  Topical treatments. You might be prescribed creams or gels to soothe your skin and reduce pain during a flare-up. This normally includes creams that are lidocaine- or capsaicin-based. […] Oral medications. There are a variety of oral medications that can help with erythromelalgia. This might include anti-inflammatory medications, nerve pain medications, aspirin, or antidepressants. Depending on the underlying cause of your erythromelalgia, beta-blockers or other blood pressure medications might also help reduce flare-ups. […] IV medications. Your doctor might recommend IV medication treatment if your erythromelalgia flare-ups are severe or arent controlled by topical or oral treatments. Youll generally receive a single IV treatment. […] Treatment for erythromelalgia depends on the cause and severity of your condition and how you respond to initial treatments.

• #45 Erythomelalgia | SRUK

  https://www.sruk.co.uk/scleroderma/erythomelalgia/

  Can it be treated? […] Intravenous infusion In some cases, when pain has not been controlled by medication taken by mouth (orally), an intravenous infusion (when medicine is given directly into your bloodstream via a drip) may be used. Lidocaine a local anaesthetic that blocks sodium channels and can help nerve-related pain may be given this way, but how long it works for varies. Your doctor will explain the procedure to you and how you should prepare for it. […] Medication taken by mouth A number of different medications have shown potential in relieving symptoms, although no single drug helps everyone. Different treatments often need to be tried under the supervision of experienced clinicians, and combinations of different medicines are sometimes needed. Your doctor will discuss treatment options with you, as this will also depend on the type of erythromelalgia you have.

• #46 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, nitroglycerin, gabapentin, typhoid vaccine, and topical oxymetazoline appear in the literature, as well as case series with intravenous bupivacaine, and lidocaine plus mexiletine. […] Medications that affect voltage-gated sodium channels (eg, lidocaine and its oral form, mexiletine) show promise. […] Prostacyclin may provide some benefit. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analogue) improved symptoms. […] A double-blind, crossover, placebo-compared study of the prostaglandin E1 analog misoprostol in 21 patients with erythromelalgia found that it reduces symptoms and microvascular arteriovenous shunting. […] Topical therapy has been studied, including transdermal lidocaine.

• #47 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, nitroglycerin, gabapentin, typhoid vaccine, and topical oxymetazoline appear in the literature, as well as case series with intravenous bupivacaine, and lidocaine plus mexiletine. […] Medications that affect voltage-gated sodium channels (eg, lidocaine and its oral form, mexiletine) show promise. […] Prostacyclin may provide some benefit. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analogue) improved symptoms. […] A double-blind, crossover, placebo-compared study of the prostaglandin E1 analog misoprostol in 21 patients with erythromelalgia found that it reduces symptoms and microvascular arteriovenous shunting. […] Topical therapy has been studied, including transdermal lidocaine.

• #48 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, nitroglycerin, gabapentin, typhoid vaccine, and topical oxymetazoline appear in the literature, as well as case series with intravenous bupivacaine, and lidocaine plus mexiletine. […] Medications that affect voltage-gated sodium channels (eg, lidocaine and its oral form, mexiletine) show promise. […] Prostacyclin may provide some benefit. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analogue) improved symptoms. […] A double-blind, crossover, placebo-compared study of the prostaglandin E1 analog misoprostol in 21 patients with erythromelalgia found that it reduces symptoms and microvascular arteriovenous shunting. […] Topical therapy has been studied, including transdermal lidocaine.

• #49 Erythromelalgia: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/200071-overview

  Topical treatments for erythromelalgia. […] Lidocaine patch for pain of erythromelalgia: follow-up of 34 patients. […] Topically Applied Midodrine, 0.2%, an 1-Agonist, for the Treatment of Erythromelalgia. […] Topical amitriptyline combined with ketamine for the treatment of erythromelalgia: a retrospective study of 36 patients at Mayo Clinic. […] Erythromelalgia: Identification of a corticosteroid-responsive subset. […] Secondary Erythromelalgia Successfully Treated With Intravenous Immunoglobulin. […] Treatment of Na(v)1.7-mediated pain in inherited erythromelalgia using a novel sodium channel blocker. […] Prostacyclin reduces symptoms and sympathetic dysfunction in erythromelalgia in a double-blind randomized pilot study. […] The prostaglandin E1 analog misoprostol reduces symptoms and microvascular arteriovenous shunting in erythromelalgia-a double-blind, crossover, placebo-compared study.

• #50 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Various classes of antidepressants have been used. […] Gabapentinoids such as gabapentin and pregabalin are commonly used to treat neuropathic pain. […] Antihistamines may have potent vascular effects and may be considered for treatment. […] Procedural interventions have been reported, and range from less-invasive techniques such as injection of botulinum toxin and sympathetic blockade to highly invasive procedures such as implantation of spinal cord stimulators. […] The efficacy of an intensive pain rehabilitation program has been evaluated in eight adults with EM. […] There has been substantial advancement in the understanding of the pathophysiology underlying EM. […] Most patients with EM require multimodal pharmacotherapy, specifically targeting both pathophysiology and neuropathic pain. […] A common starting point would be to trial agents with least risk and most likely efficacy. […] Finally, physical and occupational therapy, rehabilitation, and pain psychology treatment would also be recommended.

• #51 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. The pain is often very severe, and treating erythromelalgia is frustrating and difficult. […] A bilateral sympathetic block with lidocaine and triamcinolone resulted in relief from the pain. 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.

• #52 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. The pain is often very severe, and treating erythromelalgia is frustrating and difficult. […] A bilateral sympathetic block with lidocaine and triamcinolone resulted in relief from the pain. 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.

• #53 Sympathetic Block for Treating Primary Erythromelalgia

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

  The invasive procedures for erythromelalgia include epidural block, sympathetic ganglion block, spinal cord stimulator insertion and brachial plexus block. […] In our patient’s case, we used steroid when we performed a caudal epidural block and a sympathetic ganglion block. […] A sympathetic block may be an effective for those cases that are refractory to non-invasive treatment.

• #54 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Alternative therapies used rarely for refractory cases are epidural infusions of bupivacaine/ropivacaine, transcranial magnetic stimulation, subcutaneous injection of botulinum toxin A, and thoracic or lumbar sympathectomy. […] Some studies postulate that pain rehabilitation programs and patient counseling can improve physical and emotional functioning in a patient with erythromelalgia. […] 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. […] Try the next step only after the failure of previous steps and treat underlying myeloproliferative disorder if present.

• #55 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. Here we present a case of a 34-year-old female with erythromelalgia who we treated successfully with a lumbar epidural infusion of ropivacaine and fentanyl. The patient had complete relief shortly after the epidural infusion, and she remained stable with only minor pain two weeks and nine months later. With this case, we have reviewed the interventional treatments of erythromelalgia. 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. […] Given her classic presentation for erythromelalgia, the pain service started the full dose of aspirin because significant subpopulation of erythromelalgia patients are responsive to aspirin. However, she was unresponsive to aspirin and still reported her pain as 10/10. We subsequently placed a lumbar epidural and ran a continuous infusion of 0.0625% ropivacaine with 2 mcg/ml fentanyl at 12 ml/hour. Her pain score decreased to 0-1/10 almost immediately after the initiation of epidural infusion.

• #56 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Alternative therapies used rarely for refractory cases are epidural infusions of bupivacaine/ropivacaine, transcranial magnetic stimulation, subcutaneous injection of botulinum toxin A, and thoracic or lumbar sympathectomy. […] Some studies postulate that pain rehabilitation programs and patient counseling can improve physical and emotional functioning in a patient with erythromelalgia. […] 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. […] Try the next step only after the failure of previous steps and treat underlying myeloproliferative disorder if present.

• #57 Surgical treatment of a patient with erythromelalgia (Mitchell’s syndrome) using invasive spinal cord stimulation: A Clinical case – Toriya – Pediatric Traumatology, Orthopaedics and Reconstructive Surgery

  https://journals.eco-vector.com/turner/article/view/108045

  Erythromelalgia is a rare hereditary disorder manifesting the basic triad of symptoms: erythro redness, melos limb, and algos pain. Currently, etiotropic therapy for erythromelalgia that demonstrates high efficacy in individuals with this pathology, has not been developed. Moreover, there is no consensus on treatment strategies for this category of patients, emphasized by the absence of clinical guidelines for the treatment of erythromelalgia. Treatment of patients with erythromelalgia is currently based on sequential pharmacotherapy in order to select the most effective therapy. […] For the first time in Russia, a technique of invasive spinal cord stimulation was used in a pediatric patient with erythromelalgia, which resulted in a significant reduction of neuropathic pain, restoration of vasomotor regulation in the form of reduced edema and hyperemia.

• #58 Surgical treatment of a patient with erythromelalgia (Mitchell’s syndrome) using invasive spinal cord stimulation: A Clinical case – Toriya – Pediatric Traumatology, Orthopaedics and Reconstructive Surgery

  https://journals.eco-vector.com/turner/article/view/108045

  In a patient with prolonged and pronounced refractory neuropathic pain caused by erythromelalgia, spinal cord stimulation was the only effective treatment technique alternative to symptomatic and drug therapy. Spinal cord stimulation should be considered as a method of treating neuropathic pain associated with pharmacoresistant forms of erythromelalgia. […] The spinal cord stimulator implantation was part of a multimodal treatment regimen for a patient with this complex and debilitating disease based on the lack of effect from long-term symptomatic therapy and the deterioration of the child’s condition. Regression of neuropathic pain syndrome and dyscirculatory disorders was achieved as a result of surgical treatment in the immediate postoperative period. Thus, spinal cord stimulation has become the only effective treatment method, an alternative to symptomatic and drug therapy, in a patient with prolonged and severe refractory neuropathic pain caused by erythromelalgia. Spinal cord stimulation should be considered as one of the treatment methods for neuropathic pain associated with drug-resistant forms of erythromelalgia.

• #59 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Alternative therapies used rarely for refractory cases are epidural infusions of bupivacaine/ropivacaine, transcranial magnetic stimulation, subcutaneous injection of botulinum toxin A, and thoracic or lumbar sympathectomy. […] Some studies postulate that pain rehabilitation programs and patient counseling can improve physical and emotional functioning in a patient with erythromelalgia. […] 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. […] Try the next step only after the failure of previous steps and treat underlying myeloproliferative disorder if present.

• #60 Erythromelalgia

  https://dermnetnz.org/topics/erythromelalgia

  Topical capsaicin cream has been reported with varying results. […] Aspirin relieves symptoms of erythromelalgia related to thrombocytosis. Sodium channel blockers mexiletine has been shown to improve pain, especially in patients with V872G mutation. Calcium antagonists nifedipine, diltiazem. Prostaglandins misoprostol. Intravenous infusions of nitroprusside, lignocaine (lidocaine) and prostaglandin. Medications used for neuropathic pain include serotonin re-uptake inhibitors venlafaxine, sertraline, fluoxetine, paroxetine, tricyclic antidepressants amitriptyline, imipramine, and anticonvulsants gabapentin, carbamazepine. […] Several drugs are undergoing clinical trials in primary erythromelalgia. Examples include Nav1.7 channel modulators (PF-05089771) and NAV1.7 channel blockers (TV-45070). […] Surgical sympathectomy (a procedure in which the sympathetic nerve fibres are selectively cut) includes thoracic sympathectomy for hands and lumbar sympathectomy for feet. Patients respond quite variably and no single therapy has proved consistently effective.

• #61 Erythromelalgia: A Very Rare Condition

  https://www.verywellhealth.com/erythromelalgia-overview-4582735

  Erythromelalgia Treatment, therapy […] Treatment options should follow a stepwise approach that begins with nonpharmacological measures, followed by topical medicines, oral medications, pain rehabilitation programs, and, in extreme cases, surgery. It may take some time to find a treatment plan that works. […] There is no single treatment for EM, and there is also no cure. In secondary EM, treating the underlying condition may bring about relief. Most of the time, however, EM cannot be fully treated. In these situations, symptom relief is the goal. […] Common treatments used to control EM symptoms: Aspirin, Carbamazepine, Gabapentin, Sodium nitroprusside, Nitroglycerin, Antihistamines, Sodium ion channel blockers, Prostaglandins. […] These medications can be used in combination as needed. Other less commonly used treatments have provided relief, such as itraconazole, an antifungal. […] Cooling the affected areas with cold packs and elevating flaring feet can help with coping. […] Therapies such as biofeedback and cognitive behavioral therapy may work for some people. […] Nerve blocks are among the treatments that are sometimes used. In some cases, repeat nerve blocks may be needed, but the condition can resolve after just one treatment. […] Surgery is considered when no other treatments provide relief. A surgical procedure, called a sympathectomy, cuts nerve endings that transmit pain signals to the hands and feet. There are serious risks associated with this surgery, including bleeding, infection, and nerve damage. Doctors will consider sympathectomy only when a person’s quality of life is significantly affected by EM. […] Though there is no cure for EM, the outlook can still be positive. Treatment may bring about complete relief of symptoms. Most of the time, however, EM cannot be fully treated. Treatment takes some trial and error to find a plan that works to relieve symptoms.

• #62 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  Evaluation and treatment can be conducted in an outpatient setting. Treatment is primarily medical and supportive. Local measures, such as cooling or elevating the extremity, may relieve symptoms. Avoid excessive warming or dependency of the extremity. The environment should be modified so that it is not too hot. […] A number of topical and systemic medications have been used for treatment of erythromelalgia. As current therapies provide only symptomatic relief, topical medications are generally preferred, in view of their more limited adverse effects. […] Case reports describe successful use of spinal cord stimulation and ganglion stimulation and blocks. […] With secondary erythromelalgia, treatment of the underlying disease process may lead to improvement or resolution of the erythromelalgia.

• #63 What Is Erythromelalgia?

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

  Erythromelalgia Treatment […] There are many treatments for erythromelalgia, but they are dependent upon the cause. […] For those with secondary erythromelalgia, it may be helpful to treat the underlying disease causing erythromelalgia. […] For patients with primary erythromelalgia, medication is usually prescribed. These medications are usually oral and can be successful in relieving symptoms associated with erythromelalgia. […] These medications include: Gabapentin, carbamazepine, and other anticonvulsants […] Amitriptyline, venlafaxine, and other antidepressants […] Cetirizine, diphenhydramine, and other antihistamines […] Aspirin […] Certain blood pressure medications […] Prescription painkillers. […] Other treatment methods include: Lidocaine injections received via a vein located in your arm

• #64 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Some studies have shown that topical lidocaine patches, compounded topical amitriptyline-ketamine, and topical capsaicin applied three times daily may improve the pain associated with erythromelalgia. While 0.2% midodrine compounded in a moisturizing cream applied thrice a day might improve redness associated with erythromelalgia. Topical therapy should be continued for two to four weeks to assess the efficacy. […] Aspirin is the drug of choice for erythromelalgia associated with thrombocytopenia or myeloproliferative disorder. Other NSAIDs like anagrelide may be used as an alternative. Diagnosing and treating the underlying myeloproliferative disorder is of utmost importance for the improvement of erythromelalgia. In this patient population, the addition of hydroxyurea (chemotherapy) to reduce the platelet count may also improve the symptoms of erythromelalgia, while phlebotomy might be useful for patients with polycythemia vera.

• #65 Erythromelalgia Treatment & Management: Approach Considerations, Pharmacologic Therapy

  https://emedicine.medscape.com/article/200071-treatment

  In patients with myeloproliferative disorders, chemotherapy to reduce the platelet count often alleviates symptoms, but it is not universally effective. Some patients with polycythemia vera have responded to phlebotomy. A hematologist may be consulted if an associated myeloproliferative disorder is present. […] Surgery probably has no role in the management of erythromelalgia except to treat the rare complication of gangrene. Surgical sympathectomy has been attempted, with variable results. […] For primary erythromelalgia, the best therapy is unknown. A wide range of agents has been studied, including the following: Vascular agents – Aspirin, prostaglandins; Sodium channel blockers – Lidocaine, mexiletine, carbamazepine, oxcarbazepine; Calcium channel blockers – Amlodipine, nifedipine, diltiazem, high-dose oral magnesium; Antidepressants – Tricyclic antidepressants, serotonin reuptake inhibitors; Anticonvulsants – Gabapentin, pregabalin; Antihistamines; Immunosuppressants.

• #66 Erythromelalgia

  https://www.pcds.org.uk/clinical-guidance/erythromelalgia

  Erythromelalgia presents before the appearance of the myeloproliferative disorder in 85% of cases and by a median time of 2.5 years. Aspirin has been shown to dramatically relieve symptoms of erythromelalgia secondary to myeloproliferative disorders, but not other causes of secondary erythromelalgia […] Except for cases secondary to myeloproliferative disorders, erythromelalgia is a very difficult condition to treat, several medications may need to be tried with variable response and there is lack of evidence for the treatment ladder including which agent to use first. […] Manage any underlying cause […] Aspirin dramatically relieves symptoms of erythromelalgia secondary to myeloproliferative disorders, but not other causes of secondary erythromelalgia […] Mexiletine, a non-selective sodium channel blocker has been found to be effective in some cases of primary erythromelalgia.

• #67 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Some studies have shown that topical lidocaine patches, compounded topical amitriptyline-ketamine, and topical capsaicin applied three times daily may improve the pain associated with erythromelalgia. While 0.2% midodrine compounded in a moisturizing cream applied thrice a day might improve redness associated with erythromelalgia. Topical therapy should be continued for two to four weeks to assess the efficacy. […] Aspirin is the drug of choice for erythromelalgia associated with thrombocytopenia or myeloproliferative disorder. Other NSAIDs like anagrelide may be used as an alternative. Diagnosing and treating the underlying myeloproliferative disorder is of utmost importance for the improvement of erythromelalgia. In this patient population, the addition of hydroxyurea (chemotherapy) to reduce the platelet count may also improve the symptoms of erythromelalgia, while phlebotomy might be useful for patients with polycythemia vera.

• #68 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Some studies have shown that topical lidocaine patches, compounded topical amitriptyline-ketamine, and topical capsaicin applied three times daily may improve the pain associated with erythromelalgia. While 0.2% midodrine compounded in a moisturizing cream applied thrice a day might improve redness associated with erythromelalgia. Topical therapy should be continued for two to four weeks to assess the efficacy. […] Aspirin is the drug of choice for erythromelalgia associated with thrombocytopenia or myeloproliferative disorder. Other NSAIDs like anagrelide may be used as an alternative. Diagnosing and treating the underlying myeloproliferative disorder is of utmost importance for the improvement of erythromelalgia. In this patient population, the addition of hydroxyurea (chemotherapy) to reduce the platelet count may also improve the symptoms of erythromelalgia, while phlebotomy might be useful for patients with polycythemia vera.

• #69 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Some studies have shown that topical lidocaine patches, compounded topical amitriptyline-ketamine, and topical capsaicin applied three times daily may improve the pain associated with erythromelalgia. While 0.2% midodrine compounded in a moisturizing cream applied thrice a day might improve redness associated with erythromelalgia. Topical therapy should be continued for two to four weeks to assess the efficacy. […] Aspirin is the drug of choice for erythromelalgia associated with thrombocytopenia or myeloproliferative disorder. Other NSAIDs like anagrelide may be used as an alternative. Diagnosing and treating the underlying myeloproliferative disorder is of utmost importance for the improvement of erythromelalgia. In this patient population, the addition of hydroxyurea (chemotherapy) to reduce the platelet count may also improve the symptoms of erythromelalgia, while phlebotomy might be useful for patients with polycythemia vera.

• #70 Erythromelalgia: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/200071-overview

  Erythromelalgia is a rare disorder characterized by burning pain, warmth, and redness, predominantly involving the extremities. […] There is no known cure for erythromelalgia and thus symptomatic treatment forms the cornerstone of management. Therapies that are effective for many patients include aspirin, topical agents, and behavioral modifications. […] Patients who respond to aspirin have little morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally is unremitting. […] Patients with erythromelalgia require education about the etiology, manifestations, and possible triggers of the disease. […] Follow-up with a provider familiar with erythromelalgia is important. An annual complete blood count with differential should be performed to monitor for signs of a developing myeloproliferative disorder.

• #71 Erythromelalgia

  https://www.pcds.org.uk/clinical-guidance/erythromelalgia

  Erythromelalgia presents before the appearance of the myeloproliferative disorder in 85% of cases and by a median time of 2.5 years. Aspirin has been shown to dramatically relieve symptoms of erythromelalgia secondary to myeloproliferative disorders, but not other causes of secondary erythromelalgia […] Except for cases secondary to myeloproliferative disorders, erythromelalgia is a very difficult condition to treat, several medications may need to be tried with variable response and there is lack of evidence for the treatment ladder including which agent to use first. […] Manage any underlying cause […] Aspirin dramatically relieves symptoms of erythromelalgia secondary to myeloproliferative disorders, but not other causes of secondary erythromelalgia […] Mexiletine, a non-selective sodium channel blocker has been found to be effective in some cases of primary erythromelalgia.

• #72 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Various classes of antidepressants have been used. […] Gabapentinoids such as gabapentin and pregabalin are commonly used to treat neuropathic pain. […] Antihistamines may have potent vascular effects and may be considered for treatment. […] Procedural interventions have been reported, and range from less-invasive techniques such as injection of botulinum toxin and sympathetic blockade to highly invasive procedures such as implantation of spinal cord stimulators. […] The efficacy of an intensive pain rehabilitation program has been evaluated in eight adults with EM. […] There has been substantial advancement in the understanding of the pathophysiology underlying EM. […] Most patients with EM require multimodal pharmacotherapy, specifically targeting both pathophysiology and neuropathic pain. […] A common starting point would be to trial agents with least risk and most likely efficacy. […] Finally, physical and occupational therapy, rehabilitation, and pain psychology treatment would also be recommended.

• #73 Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review | Pediatric Research

  https://www.nature.com/articles/s41390-025-03817-4

  The most common pharmacological classes included adrenergic agonists, antihistamines, beta-blockers, calcium channel blockers, corticosteroids, cyclooxygenase (COX) inhibitors, opioid receptor agonists, sodium channel blocker, and selective serotonin/serotonin-norepinephrine reuptake inhibitors (SSRIs/SNRIs). […] Our scoping review highlights significant co-morbidities in youth with erythromelalgia which include anxiety, depression, suicidal ideation, and sleep impairment, alongside physical and social limitations. […] Referral to a comprehensive multidisciplinary pain rehabilitation center could be considered for youth with severe, refractory, or disabling EM.

• #74 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Alternative therapies used rarely for refractory cases are epidural infusions of bupivacaine/ropivacaine, transcranial magnetic stimulation, subcutaneous injection of botulinum toxin A, and thoracic or lumbar sympathectomy. […] Some studies postulate that pain rehabilitation programs and patient counseling can improve physical and emotional functioning in a patient with erythromelalgia. […] 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. […] Try the next step only after the failure of previous steps and treat underlying myeloproliferative disorder if present.

• #75 Current pain management strategies for patients with erythromelalgia: | JPR

  https://www.dovepress.com/current-pain-management-strategies-for-patients-with-erythromelalgia-a-peer-reviewed-fulltext-article-JPR

  Many pharmacologic agents have been used to treat EM over the decades. […] Aspirin has been considered as a first-line treatment, likely due to early studies reporting its efficacy and the low side effect profile. […] There appears to be a limited role for nonsteroidal anti-inflammatory drugs other than aspirin in the treatment of EM. […] Sodium channel blockers have proved promising in the preclinical setting. […] Positive response to lidocaine infusions has been considered as an indicator of success with mexiletine, an orally available form of lidocaine. […] Most patients with EM require multimodal pharmacotherapy, specifically targeting both pathophysiology and neuropathic pain. […] A common starting point would be to trial agents with least risk and most likely efficacy. […] Finally, physical and occupational therapy, rehabilitation, and pain psychology treatment would also be recommended.

• #76

  https://ijtmb.org/index.php/ijtmb/article/download/83/140?inline=1

  Erythromelalgia is characterized by temperature-dependent redness, pain, and warmth in one or more extremities. It may be a primary disease, or it may occur secondarily because of underlying illness. It is a chronic, debilitating condition often resistant to medical treatment. […] The present report evaluates massage as a complementary therapy to reduce pain and other symptoms associated with erythromelalgia. […] Massage therapy was introduced over the course of 9 treatments, each 1 hour in duration, using various massage therapy techniques, remedial exercise, and recommended home care. […] In this patient with erythromelalgia, effleurage and petrissage as massage therapy techniques provided temporary pain relief in the lower extremities and long-term benefits that relieved anxiety, which improved restorative sleep and increased the patients participation in activities of daily living.

• #77

  https://ijtmb.org/index.php/ijtmb/article/download/83/140?inline=1

  For this treatment protocol, therapist observation and patient feedback suggest that massage therapy may lead to a state of increased relaxation, decreased stress, decreased muscle tension, and improved sleep. These positive effects may have an indirect role in the ability of the patient to cope with erythromelalgia day to day. […] The long-term goals of MT were to reduce the severity of erythromelalgia symptoms in the lower extremities, reduce pain, increase relaxation, improve circulatory flow, improve restorative sleep, and enhance quality of life. […] The goal in this case was to introduce MT techniques as a method of pain management for symptoms of erythromelalgia. […] Our results reveal that relaxation massage techniques, such as superficial effleurage to the upper body and extremities, provided restorative sleep and helped to reduce anxiety for the patient.

• #78 Erythromelalgia

  https://www.nhs.uk/conditions/erythromelalgia/

  Erythromelalgia is a rare condition that causes burning pain, redness and hot skin, mainly in the feet. It can be constant or come and go, but treatment and avoiding triggers can help. […] How erythromelalgia is treated depends on the cause. […] Most treatment aims to prevent flare-ups. It can include medicines, creams, gels, sprays, tablets and medicines given into a vein. […] Erythromelalgia can be difficult to treat. You may need to try different combinations of treatments to find the ones that work for you. […] Living with pain can be difficult. If your symptoms are affecting your mental health, cognitive behavioural therapy and counselling can help. […] There are things you can try to help control erythromelalgia symptoms and avoid flare-ups.

• #79

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

  Treatment of erythromelalgia with propranolol. […] Successful treatment of erythromelalgia with sodium nitroprusside. […] Lidocaine patch for pain of erythromelalgia. […] Lidocaine and mexiletine therapy for erythromelalgia. […] Resolution of refractory symptoms of secondary erythermalgia with intermittent epidural bupivacaine. […] Continuous epidural infusion of bupivacaine and fentanyl for erythromelalgia in an adolescent. […] Treatment of primary erythromelalgia with cyproheptadine. […] Treatment of primary erythromelalgia with piroxicam. […] Successful treatment of erythromelalgia with intrathecal hydromorphone and clonidine. […] Treatment of erythromelalgia with a serotonin/noradrenaline reuptake inhibitor. […] Alleviation of erythromelalgia with venlafaxine. […] Gabapentin for the treatment of familial erythromelalgia pain.

• #80 Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review | Pediatric Research

  https://www.nature.com/articles/s41390-025-03817-4

  The most common pharmacological classes included adrenergic agonists, antihistamines, beta-blockers, calcium channel blockers, corticosteroids, cyclooxygenase (COX) inhibitors, opioid receptor agonists, sodium channel blocker, and selective serotonin/serotonin-norepinephrine reuptake inhibitors (SSRIs/SNRIs). […] Our scoping review highlights significant co-morbidities in youth with erythromelalgia which include anxiety, depression, suicidal ideation, and sleep impairment, alongside physical and social limitations. […] Referral to a comprehensive multidisciplinary pain rehabilitation center could be considered for youth with severe, refractory, or disabling EM.

• #81 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia treatment often includes drugs you take by mouth. Many of these drugs are for other medical conditions. But they can be effective in relieving erythromelalgia symptoms. […] The treatment thats right for you depends on the cause. For secondary erythromelalgia, treating the underlying issue may relieve your symptoms. Treatment for primary erythromelalgia includes medications. […] Erythromelalgia treatment may also include: Lidocaine infusions, a pain medication you receive through a vein in your arm. Nutritional supplements, such as magnesium and alpha-lipoic acid. Pain management therapies, including nerve blocks. Topical medications, such as creams you apply to your skin. […] Nonmedical therapies include: Elevating the affected area. Resting on a cool surface, such as a tile floor. Using a fan. Wrapping cool first-aid gel packs in a thin towel and placing them on your skin.

• #82 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia treatment often includes drugs you take by mouth. Many of these drugs are for other medical conditions. But they can be effective in relieving erythromelalgia symptoms. […] The treatment thats right for you depends on the cause. For secondary erythromelalgia, treating the underlying issue may relieve your symptoms. Treatment for primary erythromelalgia includes medications. […] Erythromelalgia treatment may also include: Lidocaine infusions, a pain medication you receive through a vein in your arm. Nutritional supplements, such as magnesium and alpha-lipoic acid. Pain management therapies, including nerve blocks. Topical medications, such as creams you apply to your skin. […] Nonmedical therapies include: Elevating the affected area. Resting on a cool surface, such as a tile floor. Using a fan. Wrapping cool first-aid gel packs in a thin towel and placing them on your skin.

• #83 What Is Erythromelalgia?

  https://www.icliniq.com/articles/skin-care/erythromelalgia

  Topical medications can also be given. […] Systemic infusion of Gabapentin or Venlafaxine for two to three months. […] Rehabilitation programs. […] Patient education is done. […] Regular follow-up. […] Capsaicin cream. […] Doxepin cream. […] Oral Medications: Aspirin. […] Venlafaxine. […] Sertraline. […] Tramadol. […] Tricyclic antidepressants(TCA) include Amitriptyline and Imipramine. […] Anticonvulsants: Gabapentin and Carbamazepine. […] Calcium antagonists: Nifedipine. […] Misoprostol. […] Parenteral Approach: Nitroprusside infusions. […] Lidocaine infusions. […] Prostaglandin infusions. […] Invasive Approach: Sympathetic blocks and epidurals. […] Sympathectomies. […] Dorsal column stimulator. […] Neurosurgery. […] Non-medical Approach for Erythromelalgia: Hypnosis. […] Biofeedback. […] Acupuncture.

• #84 What Is Erythromelalgia?

  https://www.icliniq.com/articles/skin-care/erythromelalgia

  Topical medications can also be given. […] Systemic infusion of Gabapentin or Venlafaxine for two to three months. […] Rehabilitation programs. […] Patient education is done. […] Regular follow-up. […] Capsaicin cream. […] Doxepin cream. […] Oral Medications: Aspirin. […] Venlafaxine. […] Sertraline. […] Tramadol. […] Tricyclic antidepressants(TCA) include Amitriptyline and Imipramine. […] Anticonvulsants: Gabapentin and Carbamazepine. […] Calcium antagonists: Nifedipine. […] Misoprostol. […] Parenteral Approach: Nitroprusside infusions. […] Lidocaine infusions. […] Prostaglandin infusions. […] Invasive Approach: Sympathetic blocks and epidurals. […] Sympathectomies. […] Dorsal column stimulator. […] Neurosurgery. […] Non-medical Approach for Erythromelalgia: Hypnosis. […] Biofeedback. […] Acupuncture.

• #85 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Many pharmacologic agents have been used to treat EM over the decades. […] Aspirin has been considered as a first-line treatment, likely due to early studies reporting its efficacy and the low side effect profile. […] There appears to be a limited role for nonsteroidal anti-inflammatory drugs other than aspirin in the treatment of EM. […] Prostaglandin and prostacyclin analogs with vasodilatory effects have been the subjects of two small RCTs in EM treatment of pain. […] Drugs that target the underlying genetic cause of cases of hereditary EM (Nav1.7 sodium channel) have proved promising in the preclinical setting. […] Lidocaine and mexiletine provide nonselective partial blockade of voltage-gated sodium channels, working on segment 6 of domain IV of the alpha subunit to shorten the action potential duration.

• #86 Erythromelalgia

  https://dermnetnz.org/topics/erythromelalgia

  Topical capsaicin cream has been reported with varying results. […] Aspirin relieves symptoms of erythromelalgia related to thrombocytosis. Sodium channel blockers mexiletine has been shown to improve pain, especially in patients with V872G mutation. Calcium antagonists nifedipine, diltiazem. Prostaglandins misoprostol. Intravenous infusions of nitroprusside, lignocaine (lidocaine) and prostaglandin. Medications used for neuropathic pain include serotonin re-uptake inhibitors venlafaxine, sertraline, fluoxetine, paroxetine, tricyclic antidepressants amitriptyline, imipramine, and anticonvulsants gabapentin, carbamazepine. […] Several drugs are undergoing clinical trials in primary erythromelalgia. Examples include Nav1.7 channel modulators (PF-05089771) and NAV1.7 channel blockers (TV-45070). […] Surgical sympathectomy (a procedure in which the sympathetic nerve fibres are selectively cut) includes thoracic sympathectomy for hands and lumbar sympathectomy for feet. Patients respond quite variably and no single therapy has proved consistently effective.

• #87 Erythromelalgia

  https://dermnetnz.org/topics/erythromelalgia

  Topical capsaicin cream has been reported with varying results. […] Aspirin relieves symptoms of erythromelalgia related to thrombocytosis. Sodium channel blockers mexiletine has been shown to improve pain, especially in patients with V872G mutation. Calcium antagonists nifedipine, diltiazem. Prostaglandins misoprostol. Intravenous infusions of nitroprusside, lignocaine (lidocaine) and prostaglandin. Medications used for neuropathic pain include serotonin re-uptake inhibitors venlafaxine, sertraline, fluoxetine, paroxetine, tricyclic antidepressants amitriptyline, imipramine, and anticonvulsants gabapentin, carbamazepine. […] Several drugs are undergoing clinical trials in primary erythromelalgia. Examples include Nav1.7 channel modulators (PF-05089771) and NAV1.7 channel blockers (TV-45070). […] Surgical sympathectomy (a procedure in which the sympathetic nerve fibres are selectively cut) includes thoracic sympathectomy for hands and lumbar sympathectomy for feet. Patients respond quite variably and no single therapy has proved consistently effective.

• #88 A new study raises hopes for a potential treatment for erythromelalgia | FAU Erlangen-Nürnberg

  https://www.fau.eu/2023/11/news/research/a-new-study-raises-hopes-for-a-potential-treatment-for-erythromelalgia/

  Research project led by Erlangen Department of Dermatology launched […] Until now there has been no effective treatment. The new EASE study (Efficacy of ATX01 Study in Erythromelalgia) hopes to change that. […] As part of the study, the first patients have already received ATX01, an active agent produced by the biotechnology company Algo Therapeutix. The effectiveness of the medicine is now being tested. ATX01 is applied externally. It is intended to block pain-conducting sodium channels, thereby soothing the at times excruciating pain of erythromelalgia. There is currently no medication available to treat the disease. We are therefore very interested to see the results of the study, which should be available in the first half of 2024, Dr. Bauerschmitz explains.

• #89 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Alternative therapies used rarely for refractory cases are epidural infusions of bupivacaine/ropivacaine, transcranial magnetic stimulation, subcutaneous injection of botulinum toxin A, and thoracic or lumbar sympathectomy. […] Some studies postulate that pain rehabilitation programs and patient counseling can improve physical and emotional functioning in a patient with erythromelalgia. […] 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. […] Try the next step only after the failure of previous steps and treat underlying myeloproliferative disorder if present.

• #90 Erythromelalgia: A Very Rare Condition

  https://www.verywellhealth.com/erythromelalgia-overview-4582735

  Erythromelalgia Treatment, therapy […] Treatment options should follow a stepwise approach that begins with nonpharmacological measures, followed by topical medicines, oral medications, pain rehabilitation programs, and, in extreme cases, surgery. It may take some time to find a treatment plan that works. […] There is no single treatment for EM, and there is also no cure. In secondary EM, treating the underlying condition may bring about relief. Most of the time, however, EM cannot be fully treated. In these situations, symptom relief is the goal. […] Common treatments used to control EM symptoms: Aspirin, Carbamazepine, Gabapentin, Sodium nitroprusside, Nitroglycerin, Antihistamines, Sodium ion channel blockers, Prostaglandins. […] These medications can be used in combination as needed. Other less commonly used treatments have provided relief, such as itraconazole, an antifungal. […] Cooling the affected areas with cold packs and elevating flaring feet can help with coping. […] Therapies such as biofeedback and cognitive behavioral therapy may work for some people. […] Nerve blocks are among the treatments that are sometimes used. In some cases, repeat nerve blocks may be needed, but the condition can resolve after just one treatment. […] Surgery is considered when no other treatments provide relief. A surgical procedure, called a sympathectomy, cuts nerve endings that transmit pain signals to the hands and feet. There are serious risks associated with this surgery, including bleeding, infection, and nerve damage. Doctors will consider sympathectomy only when a person’s quality of life is significantly affected by EM. […] Though there is no cure for EM, the outlook can still be positive. Treatment may bring about complete relief of symptoms. Most of the time, however, EM cannot be fully treated. Treatment takes some trial and error to find a plan that works to relieve symptoms.

• #91 Erythromelalgia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/91838

  Alternative therapies used rarely for refractory cases are epidural infusions of bupivacaine/ropivacaine, transcranial magnetic stimulation, subcutaneous injection of botulinum toxin A, and thoracic or lumbar sympathectomy. […] Some studies postulate that pain rehabilitation programs and patient counseling can improve physical and emotional functioning in a patient with erythromelalgia. […] 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. […] Try the next step only after the failure of previous steps and treat underlying myeloproliferative disorder if present.

• #92 Erythromelalgia Clinic in Minnesota – Overview – Mayo Clinic

  https://www.mayoclinic.org/departments-centers/erythromelalgia-clinic-in-minnesota/overview/ovc-20421220

  Erythromelalgia is a rare condition that causes extreme heat, changes in color of the skin on the bottom of the feet, such as redness or darkening of the skin. […] Your care at the Erythromelalgia Clinic in Minnesota is guided by a multispecialty team of doctors, including specialists in skin conditions (dermatologists), the nervous system (neurology), and the blood and lymph system (vascular medicine). Your visit typically includes specialized testing and an assessment of your symptoms. Your doctors work together to arrive at an accurate diagnosis. They then develop a treatment plan with you that meets your needs and considers possible side effects of each treatment option.

• #93

  https://www.ijord.com/index.php/ijord/article/view/1079

  Erythromelalgia presents with redness, pain and warmth of the extremities, which is exacerbated by warming and relieved by cooling. Treatment of pain in erythromelalgia can be difficult, and often polypharmacy is utilized. No single therapy has been found to provide complete relief for any patient. Therefore, a multidisciplinary approach is required to treat pain and its significant negative impact across many domains of functioning. […] The patient required a multidisciplinary approach for pain management and surgical intervention was required due to his refractory nature. However, this also does not give complete relief and thus a combination of both medical and surgical methods is required for best control of symptoms.

• #94 Current pain management strategies for patients with erythromelalgia: a critical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6121769/

  Various classes of antidepressants have been used. […] Gabapentinoids such as gabapentin and pregabalin are commonly used to treat neuropathic pain. […] Antihistamines may have potent vascular effects and may be considered for treatment. […] Procedural interventions have been reported, and range from less-invasive techniques such as injection of botulinum toxin and sympathetic blockade to highly invasive procedures such as implantation of spinal cord stimulators. […] The efficacy of an intensive pain rehabilitation program has been evaluated in eight adults with EM. […] There has been substantial advancement in the understanding of the pathophysiology underlying EM. […] Most patients with EM require multimodal pharmacotherapy, specifically targeting both pathophysiology and neuropathic pain. […] A common starting point would be to trial agents with least risk and most likely efficacy. […] Finally, physical and occupational therapy, rehabilitation, and pain psychology treatment would also be recommended.

• #95 FAQs | The Erythromelalgia Association

  https://burningfeet.org/resources/faqs/

  How is EM treated? There is no single cure for EM. While it is true that treating an underlying disease may sometimes bring complete relief, most cases of EM cannot be fully treated. In such cases, treatment must focus on relieving symptoms with medications and other techniques. It often takes a period of trial and error to find a treatment plan that works. Treatment options include topical medications, oral medications, supplements, and surgical procedures, as well as alternative and mind/body therapies. For a detailed list of potential treatments, please see TEAs Patients Guide. […] What is being done to find a cure? TEA encourages and funds research into effective treatments for all types of EM. Though EM is rare and has not been extensively studied, research into its causes, mechanisms, and treatments continues in both academic and commercial settings. At present, there is a growing interest in developing EM treatments, due in part to the belief that a drug designed to treat EM pain could work for many other kinds of pain as well. For the most up-to-date information on advances in EM research and treatments, see TEAs In the News, Medical Articles, Research Timeline, and FootSteps Newsletters.

• #96 Current pain management strategies for patients with erythromelalgia: | JPR

  https://www.dovepress.com/current-pain-management-strategies-for-patients-with-erythromelalgia-a-peer-reviewed-fulltext-article-JPR

  There has been substantial advancement in the understanding of the pathophysiology underlying EM. However, research findings have yet to translate to clinical practice. […] The diagnosis of EM is based on evaluation of the symptom profile and exclusion of other differential diagnosis. […] The role of immunosuppressants in the treatment of EM remains unclear, with limited data and lack of clarity regarding the role of immune function in the development of EM.

• #97 Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review | Pediatric Research

  https://www.nature.com/articles/s41390-025-03817-4

  Erythromelalgia is a rare, chronic pain disorder characterized by the triad of intense burning sensation, warmth, and redness, primarily involving the hands and feet, and usually alleviated by cold and worsened by heat. […] While factors to aid early recognition and optimize management have been identified, there are also significant gaps for future research to address. Ongoing efforts to develop a multicenter registry of pediatric erythromelalgia cases, with standardized data collection and reporting, will be beneficial to establish consensus recommendations for the diagnosis and management of pediatric erythromelalgia. […] Various combinations of pharmacological and non-pharmacological treatments and related responses were reported across most studies. […] Procedural interventions were reported in 56 studies which primarily included: neural axial blockade (i.e. epidural catheters insertion), infusions, nerve blocks, sympathectomies, and transcutaneous electrical nerve stimulations. Non-pharmacological approaches included avoidance of triggers (e.g. reduce exposure to heat), physiotherapy, psychology, pain rehabilitation programs, and other treatments.

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