Materiały źródłowe

• #1 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia (ur-i-thruh-muh-lal-jah) is a rare condition in which excess blood flow causes periodic symptom flares. Its a chronic condition, meaning that it stays with you for life. Erythromelalgia symptoms, which often include burning pain, worsen over time. […] Primary erythromelalgia is isolated and not due to an underlying disease. […] Idiopathic erythromelalgia is the most common type and occurs for unknown reasons. […] Inherited erythromelalgia is due to gene mutations. The mutations, which affect pain signals, can be passed down in families. […] Secondary erythromelalgia occurs when there is an underlying blood, neurological or immunologic condition. […] Flares usually occur when you experience a sudden increase in body temperature. […] 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.

• #2 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. This peripheral channel is expressed within the dorsal root ganglion of the sympathetic ganglion neurons. This mutation leads to hyperexcitability of the nociceptive fibers causing them to fire at subthreshold stimuli. This, in turn, leads to a previously nonpainful stimulus eliciting a painful response. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning. The symptoms of patients with secondary erythromelalgia are often milder and are relieved after the treatment of the underlying disease.

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

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

  Erythromelalgia is a rare skin condition that affects the feet, hands, arms, and legs. […] The exact causes of erythromelalgia aren’t always known. In most cases of erythromelalgia, no cause is pinpointed. But there are a few known causes. […] Erythromelalgia can be a side effect of certain medications, like: bromocriptine, calcium channel blockers, cyclosporine, iodinated contrast agents, norephedrine, topical isopropanol, rosuvastatin. […] Additionally, it can sometimes be the result of another medical condition. Conditions that may cause erythromelalgia include: multiple sclerosis (MS), peripheral neuropathy and other types of nerve damage, lupus, rheumatoid arthritis, or another autoimmune condition, polycythemia vera and conditions that impact your blood. […] Genetics can also play a role. According to an older 2007 review, a mutation on the SCN9A gene that causes erythromelalgia. This gene mutation can run in families and be passed from one generation to the next. […] Erythromelalgia flare-ups are triggered by exposure to rising temperatures, like when you exercise or are in very warm climates. The condition can occur on its own or be caused by another condition.

• #4 What Is Erythromelalgia?

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

  Erythromelalgia can occur at any age. Some patients have been diagnosed with it from childhood, but others have only received a diagnosis as an adult. […] There are a few types and subtypes of erythromelalgia, each with its own causes. These types include: […] Primary erythromelalgia occurs in isolation without any underlying diseases. There are two primary types: […] Idiopathic: The most common form of erythromelalgia. It occurs with no known cause. […] Inherited: A defective gene is passed down from parent to child. […] Secondary erythromelalgia results from an underlying condition relating to neurological, blood, or immunological disorders. These conditions include: […] Lupus, multiple sclerosis, and other autoimmune disorders […] Essential thrombocythemia a disorder of the blood that makes your body develop too many platelets

• #5 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia (ur-i-thruh-muh-lal-jah) is a rare condition in which excess blood flow causes periodic symptom flares. Its a chronic condition, meaning that it stays with you for life. Erythromelalgia symptoms, which often include burning pain, worsen over time. […] Primary erythromelalgia is isolated and not due to an underlying disease. […] Idiopathic erythromelalgia is the most common type and occurs for unknown reasons. […] Inherited erythromelalgia is due to gene mutations. The mutations, which affect pain signals, can be passed down in families. […] Secondary erythromelalgia occurs when there is an underlying blood, neurological or immunologic condition. […] Flares usually occur when you experience a sudden increase in body temperature. […] 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.

• #6 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia (ur-i-thruh-muh-lal-jah) is a rare condition in which excess blood flow causes periodic symptom flares. Its a chronic condition, meaning that it stays with you for life. Erythromelalgia symptoms, which often include burning pain, worsen over time. […] Primary erythromelalgia is isolated and not due to an underlying disease. […] Idiopathic erythromelalgia is the most common type and occurs for unknown reasons. […] Inherited erythromelalgia is due to gene mutations. The mutations, which affect pain signals, can be passed down in families. […] Secondary erythromelalgia occurs when there is an underlying blood, neurological or immunologic condition. […] Flares usually occur when you experience a sudden increase in body temperature. […] 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.

• #7 What Is Erythromelalgia?

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

  Erythromelalgia can occur at any age. Some patients have been diagnosed with it from childhood, but others have only received a diagnosis as an adult. […] There are a few types and subtypes of erythromelalgia, each with its own causes. These types include: […] Primary erythromelalgia occurs in isolation without any underlying diseases. There are two primary types: […] Idiopathic: The most common form of erythromelalgia. It occurs with no known cause. […] Inherited: A defective gene is passed down from parent to child. […] Secondary erythromelalgia results from an underlying condition relating to neurological, blood, or immunological disorders. These conditions include: […] Lupus, multiple sclerosis, and other autoimmune disorders […] Essential thrombocythemia a disorder of the blood that makes your body develop too many platelets

• #8 What Is Erythromelalgia?

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

  Erythromelalgia has a genetic etiology. […] Secondary erythromelalgia has systemic causes, which include several infections, autoimmune diseases, endocrine diseases, neoplastic conditions, medications, gout, high blood pressure, multiple sclerosis, and hematological disorders like pernicious anemia, thrombotic thrombocytopenic purpura, and also by mushroom intoxication and mercury poisoning. […] The most prevalent kind, idiopathic erythromelalgia, has no known cause. […] The cause of inherited erythromelalgia is mutated genes. The mutations that impact pain signals have the potential to run in families. […] Secondary erythromelalgia happens when there is an underlying blood, neurological, or immunologic disorder. Among them are autoimmune illnesses, including multiple sclerosis and lupus.

• #9 Erythromelalgia: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/erythromelalgia/

  Erythromelalgia is a condition characterized by episodes of pain, redness, and swelling in various parts of the body, particularly the hands and feet. […] Mutations in the SCN9A gene can cause erythromelalgia. The SCN9A gene provides instructions for making one part (the alpha subunit) of a sodium channel called NaV1.7. Sodium channels transport positively charged sodium atoms (sodium ions) into cells and play a key role in a cell’s ability to generate and transmit electrical signals. […] The SCN9A gene mutations that cause erythromelalgia result in NaV1.7 sodium channels that open more easily than usual and stays open longer than normal, increasing the flow of sodium ions into nociceptors. This increase in sodium ions enhances transmission of pain signals, leading to the signs and symptoms of erythromelalgia. […] An estimated 15 percent of cases of erythromelalgia are caused by mutations in the SCN9A gene. Other cases are thought to have a nongenetic cause or may be caused by mutations in one or more as-yet unidentified genes.

• #10 Primary erythromelalgia: a review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-015-0347-1

  Primary erythromelalgia (PE ORPHA90026) is a rare autosomal dominant neuropathy characterized by the combination of recurrent burning pain, warmth and redness of the extremities. […] Genetic etiology of PE is mutations on SCN9A, the encoding gene of a voltage-gated sodium channel subtype Nav1.7. […] This review mainly focuses on PE and the causative gene SCN9A — its mutations and their effects on Nav1.7 channels electrophysiological properties. […] The causative gene for PE, SCN9A, encodes a voltage-gated sodium channel (VGSC) subtype Nav1.7. […] More than 70 mutations on SCN9A have been associated with various clinical phenotypes, among which are pain disorders including gain-of-function disorders PE and paroxysmal extreme pain disorder (PEPD) as well as loss-of-pain disorder congenital insensitivity to pain (CIP).

• #11 Erythromelalgia

  https://dermnetnz.org/topics/erythromelalgia

  Primary erythromelalgia is caused by mutations in SCN9A, which encodes for the Nav1.7 type sodium channel receptor. Mutations are responsible for familial (inherited) erythromelalgia and sporadic forms of the condition. […] Secondary erythromelalgia is commonly associated with myeloproliferative disorders. In these disorders the bone marrow produces excessive numbers of cells, e.g. polycythaemia vera (increased red cells), and essential thrombocythaemia (increased platelets). Erythromelalgia presents before the appearance of the myeloproliferative disorder in 85% of cases. Occasionally it can occur in association with paraneoplastic disorders, autoimmune neuropathies and rarely diabetes, rheumatological and infectious diseases.

• #12 Primary erythromelalgia: a review | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-015-0347-1

  To date, more than 20 mutations have been reported responsible for PE. […] The mechanisms through which hyperpolarized shifts in activation contribute to the hyperexcitability of sensory neurons are that the leftward shifts, with or without a change in steady-state inactivation, increase the overlap between activation and inactivation curves. […] The enhanced ramp currents lead to higher availability of sodium channels at RMP during activation and fast inactivation. […] Given the fact that Nav1.7 channels are expressed in small, mostly nociceptive sensory neurons, the alterations in activation, slow inactivation, deactivation and ramp currents provide explanations to the amplified pain symptoms observed in PE patients. […] Multiple studies have suggested that large shifts in activation are related to early onsets of PE and small shifts in activation are related to late onsets of PE.

• #13 Orphanet: Primary erythromelalgia

  https://www.orpha.net/en/disease/detail/90026

  Primary erythermalgia is characterized by intermittent attacks of red, warm, painful burning extremities. It spontaneously arises during early childhood and adolescence in the absence of any detectable underlying disorder. […] The gene for autosomal dominant erythermalgia, SCN9a, is located on chromosome 2q. SCN9a is a 26 exon gene encoding the voltage-gated sodium channel alpha subunit Nav1.7. This channel is found mainly in dorsal root ganglia and sympathetic ganglia neurons. All mutations detected so far are missense and lead to a gain of function by lowering the activation threshold of Nav1.7, resulting in hyperexcitability of pain signaling neurons. […] In familial cases, the disorder is inherited as an autosomal dominant trait.

• #14 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. This peripheral channel is expressed within the dorsal root ganglion of the sympathetic ganglion neurons. This mutation leads to hyperexcitability of the nociceptive fibers causing them to fire at subthreshold stimuli. This, in turn, leads to a previously nonpainful stimulus eliciting a painful response. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning. The symptoms of patients with secondary erythromelalgia are often milder and are relieved after the treatment of the underlying disease.

• #15 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #16 Erythromelalgia: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/erythromelalgia/

  Erythromelalgia is a condition characterized by episodes of pain, redness, and swelling in various parts of the body, particularly the hands and feet. […] Mutations in the SCN9A gene can cause erythromelalgia. The SCN9A gene provides instructions for making one part (the alpha subunit) of a sodium channel called NaV1.7. Sodium channels transport positively charged sodium atoms (sodium ions) into cells and play a key role in a cell’s ability to generate and transmit electrical signals. […] The SCN9A gene mutations that cause erythromelalgia result in NaV1.7 sodium channels that open more easily than usual and stays open longer than normal, increasing the flow of sodium ions into nociceptors. This increase in sodium ions enhances transmission of pain signals, leading to the signs and symptoms of erythromelalgia. […] An estimated 15 percent of cases of erythromelalgia are caused by mutations in the SCN9A gene. Other cases are thought to have a nongenetic cause or may be caused by mutations in one or more as-yet unidentified genes.

• #17 Erythromelalgia: Definition, symptoms, and management

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

  According to TEA, there are two main types of EM: […] This type of EM does not occur due to an underlying condition or illness. […] Healthcare professionals can classify primary EM as either inherited EM or idiopathic EM. […] Inherited EM passes down genetically. It occurs due to a mutation in a gene called SCN9A, which affects how nerves send pain signals to the brain. […] About 515% of EM cases occur due to this mutation. […] The most common type of primary EM is idiopathic EM, in which there is no identifiable cause. […] Secondary erythromelalgia occurs due to another illness or condition. […] The most common causes include: […] nerve damage due to injuries or other conditions, such as carpal tunnel syndrome, sciatica, and frostbite […] autoimmune diseases, including lupus, vasculitis, and rheumatoid arthritis

• #18

  https://theburydirectory.co.uk/ext-erythromelalgia

  Erythromelalgia is a rare condition that causes episodes of burning pain and redness in the feet, and sometimes the hands, arms, legs, ears and face. […] The cause of erythromelalgia is usually unknown. […] But it’s sometimes caused by another underlying medical condition or a faulty gene inherited from a parent. […] Erythromelalgia sometimes results from an underlying condition, such as: a blood disorder like polycythaemia, nerve damage for example, caused by peripheral neuropathy, multiple sclerosis (MS), an autoimmune problem such as lupus or rheumatoid arthritis, where the immune system attacks the body’s own tissues. […] It may also be caused by certain medicines. Your doctor will be able to give you more information on this. […] Some people with erythromelalgia have a faulty gene that causes the condition. […] Erythromelalgia can run in families when the faulty gene is passed down (inherited) from a parent to their child. […] The faulty gene causes changes in the way pain signals are delivered to the brain, increasing or strengthening them.

• #19 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia (ur-i-thruh-muh-lal-jah) is a rare condition in which excess blood flow causes periodic symptom flares. Its a chronic condition, meaning that it stays with you for life. Erythromelalgia symptoms, which often include burning pain, worsen over time. […] Primary erythromelalgia is isolated and not due to an underlying disease. […] Idiopathic erythromelalgia is the most common type and occurs for unknown reasons. […] Inherited erythromelalgia is due to gene mutations. The mutations, which affect pain signals, can be passed down in families. […] Secondary erythromelalgia occurs when there is an underlying blood, neurological or immunologic condition. […] Flares usually occur when you experience a sudden increase in body temperature. […] 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.

• #20 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. This peripheral channel is expressed within the dorsal root ganglion of the sympathetic ganglion neurons. This mutation leads to hyperexcitability of the nociceptive fibers causing them to fire at subthreshold stimuli. This, in turn, leads to a previously nonpainful stimulus eliciting a painful response. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning. The symptoms of patients with secondary erythromelalgia are often milder and are relieved after the treatment of the underlying disease.

• #21 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. This peripheral channel is expressed within the dorsal root ganglion of the sympathetic ganglion neurons. This mutation leads to hyperexcitability of the nociceptive fibers causing them to fire at subthreshold stimuli. This, in turn, leads to a previously nonpainful stimulus eliciting a painful response. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning. The symptoms of patients with secondary erythromelalgia are often milder and are relieved after the treatment of the underlying disease.

• #22 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Primary erythromelalgia may be idiopathic or genetic in origin. Early-onset erythromelalgia can show a familial occurrence. Adult-onset erythromelalgia is idiopathic in the majority of cases, although an inherited case has been reported. […] Hereditary erythromelalgia is an autosomal dominant disorder caused by gain-of-function mutations in SCN9A, which encodes a voltage-gated sodium channel, subtype Nav1.7. More than 20 SCN9A mutations have been reported in patients with primary erythromelalgia. […] Causes of secondary erythromelalgia include: Myeloproliferative disorders, Infection (eg, HIV, poxvirus), Medications, Mercury poisoning, Mushroom poisoning. […] Most reported cases of secondary erythromelalgia are due to myeloproliferative disorders with thrombocytosis (most commonly polycythemia vera or essential thrombocytosis).

• #23 What Is Erythromelalgia?

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

  Erythromelalgia can occur at any age. Some patients have been diagnosed with it from childhood, but others have only received a diagnosis as an adult. […] There are a few types and subtypes of erythromelalgia, each with its own causes. These types include: […] Primary erythromelalgia occurs in isolation without any underlying diseases. There are two primary types: […] Idiopathic: The most common form of erythromelalgia. It occurs with no known cause. […] Inherited: A defective gene is passed down from parent to child. […] Secondary erythromelalgia results from an underlying condition relating to neurological, blood, or immunological disorders. These conditions include: […] Lupus, multiple sclerosis, and other autoimmune disorders […] Essential thrombocythemia a disorder of the blood that makes your body develop too many platelets

• #24 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #25 What Is Erythromelalgia?

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

  Polycythemia vera a type of blood cancer […] Raynauds phenomenon a condition that affects certain body parts, making them feel numb and turn different colors […] Thrombocytopenia a condition in which the platelet count in your blood is lower than it should be […] Neuropathy damaged or defective nerves resulting in muscle weakness, numbness, tingling, and pain in affected areas.

• #26 Erythromelalgia | Thoracic Key

  https://thoracickey.com/erythromelalgia/

  Erythromelalgia is a rare condition of the extremities characterized by the triad of redness, warmth, and pain. […] It has been argued that William Harvey could have had erythromelalgia, not gout. […] Erythromelalgia has been reported in association with myeloproliferative diseases, blood disorders, drugs, infectious diseases, food ingestion (mushrooms), neoplasms, connective tissue disease, physiological conditions (pregnancy), and neuropathies. […] The relationship of many underlying disorders to erythromelalgia is sometimes unclear, and the disorder may be a coincidental comorbidity rather than an underlying disease. […] Among the reported series, the association with myeloproliferative disease seems most constant. […] Evidence of underlying myeloproliferative disease should be sought at diagnosis and subsequently. Erythromelalgia can herald the onset of underlying myeloproliferative disease.

• #27 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #28 Erythromelalgia

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

  Most cases of erythromelalgia are primary […] Primary erythromelalgia is defined as familial erythromelalgia (Weir Mitchell’s disease) and is caused by an autosomal dominant mutation in the SCN9A gene. Most cases arise in the first decade. […] Causes of secondary erythromelalgia include: The most common cause are the myeloproliferative disorders, eg polycythaemia rubra vera. Myeloproliferative disorders are a heterogeneous group of conditions characterised by cellular proliferation of one or more haematologic cell lines in the peripheral blood, distinct from acute leukaemia. 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. […] Causes of secondary erythromelalgia (above) need to excluded. […] Aspirin dramatically relieves symptoms of erythromelalgia secondary to myeloproliferative disorders, but not other causes of secondary erythromelalgia.

• #29 Erythromelalgia | Maryville, IL | Foot Health Centers

  https://www.foothealthcenters.net/services/foot-ailments/vascular-and-nerve-issues/erythromelalgia/

  If your feet become inflamed or have a burning sensation triggered by warmth, the culprit may be erthromelalgia. […] Erythromelalgia may occur as a primary or secondary disorder. […] The primary form of erythromelalgia is not associated with another condition, and the cause is usually unknown. In rare cases, the disorder is inherited, and symptoms present themselves during childhood. […] Secondary erythromelalgia is associated with an underlying condition. Myeloproliferative (bone marrow) disorders such as polycythemia vera or thrombocythemia are the most common causes of secondary erythromelalgia. However, erythromelalgia often occurs two or three years before a myeloproliferative disorder can be diagnosed. […] Secondary erythromelalgia can also be associated with other conditions including: Certain medications (i.e. bromocriptine, which is used to treat Parkinson’s disease). Diabetes mellitus. Gout. Hypertension. Lead poisoning. Lichen sclerosus. Multiple sclerosis. Rheumatoid arthritis (RA). Spinal cord disorders. Systemic lupus erythematosus (SLE). Venous insufficiency.

• #30 Erythromelalgia: Definition, symptoms, and management

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

  According to TEA, there are two main types of EM: […] This type of EM does not occur due to an underlying condition or illness. […] Healthcare professionals can classify primary EM as either inherited EM or idiopathic EM. […] Inherited EM passes down genetically. It occurs due to a mutation in a gene called SCN9A, which affects how nerves send pain signals to the brain. […] About 515% of EM cases occur due to this mutation. […] The most common type of primary EM is idiopathic EM, in which there is no identifiable cause. […] Secondary erythromelalgia occurs due to another illness or condition. […] The most common causes include: […] nerve damage due to injuries or other conditions, such as carpal tunnel syndrome, sciatica, and frostbite […] autoimmune diseases, including lupus, vasculitis, and rheumatoid arthritis

• #31 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #32 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Primary erythromelalgia may be idiopathic or genetic in origin. Early-onset erythromelalgia can show a familial occurrence. Adult-onset erythromelalgia is idiopathic in the majority of cases, although an inherited case has been reported. […] Hereditary erythromelalgia is an autosomal dominant disorder caused by gain-of-function mutations in SCN9A, which encodes a voltage-gated sodium channel, subtype Nav1.7. More than 20 SCN9A mutations have been reported in patients with primary erythromelalgia. […] Causes of secondary erythromelalgia include: Myeloproliferative disorders, Infection (eg, HIV, poxvirus), Medications, Mercury poisoning, Mushroom poisoning. […] Most reported cases of secondary erythromelalgia are due to myeloproliferative disorders with thrombocytosis (most commonly polycythemia vera or essential thrombocytosis).

• #33 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #34 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #35 Erythromelalgia | Maryville, IL | Foot Health Centers

  https://www.foothealthcenters.net/services/foot-ailments/vascular-and-nerve-issues/erythromelalgia/

  If your feet become inflamed or have a burning sensation triggered by warmth, the culprit may be erthromelalgia. […] Erythromelalgia may occur as a primary or secondary disorder. […] The primary form of erythromelalgia is not associated with another condition, and the cause is usually unknown. In rare cases, the disorder is inherited, and symptoms present themselves during childhood. […] Secondary erythromelalgia is associated with an underlying condition. Myeloproliferative (bone marrow) disorders such as polycythemia vera or thrombocythemia are the most common causes of secondary erythromelalgia. However, erythromelalgia often occurs two or three years before a myeloproliferative disorder can be diagnosed. […] Secondary erythromelalgia can also be associated with other conditions including: Certain medications (i.e. bromocriptine, which is used to treat Parkinson’s disease). Diabetes mellitus. Gout. Hypertension. Lead poisoning. Lichen sclerosus. Multiple sclerosis. Rheumatoid arthritis (RA). Spinal cord disorders. Systemic lupus erythematosus (SLE). Venous insufficiency.

• #36 Erythromelalgia: Definition, symptoms, and management

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

  multiple sclerosis […] various blood disorders, including those that involve low platelet counts and blood cancers that cause increased production of platelets or red blood cells. […] Secondary EM can also occur due to certain medications, such as some antidepressants and calcium channel blockers. In these cases, the symptoms usually go away when a person stops taking the medication.

• #37 Erythromelalgia: Definition, symptoms, and management

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

  According to TEA, there are two main types of EM: […] This type of EM does not occur due to an underlying condition or illness. […] Healthcare professionals can classify primary EM as either inherited EM or idiopathic EM. […] Inherited EM passes down genetically. It occurs due to a mutation in a gene called SCN9A, which affects how nerves send pain signals to the brain. […] About 515% of EM cases occur due to this mutation. […] The most common type of primary EM is idiopathic EM, in which there is no identifiable cause. […] Secondary erythromelalgia occurs due to another illness or condition. […] The most common causes include: […] nerve damage due to injuries or other conditions, such as carpal tunnel syndrome, sciatica, and frostbite […] autoimmune diseases, including lupus, vasculitis, and rheumatoid arthritis

• #38 Erythromelalgia: Definition, symptoms, and management

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

  multiple sclerosis […] various blood disorders, including those that involve low platelet counts and blood cancers that cause increased production of platelets or red blood cells. […] Secondary EM can also occur due to certain medications, such as some antidepressants and calcium channel blockers. In these cases, the symptoms usually go away when a person stops taking the medication.

• #39 Erythomelalgia | SRUK

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

  What causes Erythromelalgia? For most people with the condition, the cause is unknown but some underlying cause may be found such as a medical condition or faulty gene. […] Underlying causes include: Having abnormally high levels of blood cells […] Damage to the peripheral nervous system […] Multiple Sclerosis […] An autoimmune condition […] What causes flare-ups? Warm temperatures seem to be the most frequent trigger, something which is especially problematic for those with Raynaud’s. Others have found that spices and alcohol can also make the symptoms worse. […] Primary – Primary EM is not caused by an underlying disease. It includes both inherited and idiopathic EM. […] Secondary – Cases of EM that are caused by another disease or condition are called secondary EM. The most common causes of secondary EM are: Essential thrombocythemia […] Polycythemia vera […] Thrombocytopenia […] Peripheral neuropathy […] Autoimmune diseases […] Nerve damage due to injuries or other conditions […] In some cases, when the underlying condition is treated, EM symptoms will improve or resolve completely.

• #40 What Is Erythromelalgia?

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

  Polycythemia vera a type of blood cancer […] Raynauds phenomenon a condition that affects certain body parts, making them feel numb and turn different colors […] Thrombocytopenia a condition in which the platelet count in your blood is lower than it should be […] Neuropathy damaged or defective nerves resulting in muscle weakness, numbness, tingling, and pain in affected areas.

• #41 Erythromelalgia: Definition, symptoms, and management

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

  According to TEA, there are two main types of EM: […] This type of EM does not occur due to an underlying condition or illness. […] Healthcare professionals can classify primary EM as either inherited EM or idiopathic EM. […] Inherited EM passes down genetically. It occurs due to a mutation in a gene called SCN9A, which affects how nerves send pain signals to the brain. […] About 515% of EM cases occur due to this mutation. […] The most common type of primary EM is idiopathic EM, in which there is no identifiable cause. […] Secondary erythromelalgia occurs due to another illness or condition. […] The most common causes include: […] nerve damage due to injuries or other conditions, such as carpal tunnel syndrome, sciatica, and frostbite […] autoimmune diseases, including lupus, vasculitis, and rheumatoid arthritis

• #42 Erythromelalgia – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/peripheral-arterial-disorders/erythromelalgia

  Erythromelalgia is distressing paroxysmal vasodilation of small arteries in the feet and hands and, less commonly, in the face, ears, or knees; it causes burning pain, increased skin temperature, and redness. […] This rare disorder may be Primary (cause unknown). […] It may also be secondary to Diabetes mellitus, Gout, Hypertension, Lichen sclerosus, Multiple sclerosis, Myeloproliferative disorders (eg, polycythemia vera, thrombocythemia), Rheumatoid arthritis, Spinal cord disorders, Systemic lupus erythematosus, Venous insufficiency. […] Less commonly, the disorder is related to the use of some medications (eg, nifedipine, bromocriptine). […] A rare hereditary form of erythromelalgia starts at birth or during childhood.

• #43 Erythromelalgia | Maryville, IL | Foot Health Centers

  https://www.foothealthcenters.net/services/foot-ailments/vascular-and-nerve-issues/erythromelalgia/

  If your feet become inflamed or have a burning sensation triggered by warmth, the culprit may be erthromelalgia. […] Erythromelalgia may occur as a primary or secondary disorder. […] The primary form of erythromelalgia is not associated with another condition, and the cause is usually unknown. In rare cases, the disorder is inherited, and symptoms present themselves during childhood. […] Secondary erythromelalgia is associated with an underlying condition. Myeloproliferative (bone marrow) disorders such as polycythemia vera or thrombocythemia are the most common causes of secondary erythromelalgia. However, erythromelalgia often occurs two or three years before a myeloproliferative disorder can be diagnosed. […] Secondary erythromelalgia can also be associated with other conditions including: Certain medications (i.e. bromocriptine, which is used to treat Parkinson’s disease). Diabetes mellitus. Gout. Hypertension. Lead poisoning. Lichen sclerosus. Multiple sclerosis. Rheumatoid arthritis (RA). Spinal cord disorders. Systemic lupus erythematosus (SLE). Venous insufficiency.

• #44 Erythromelalgia – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/peripheral-arterial-disorders/erythromelalgia

  Erythromelalgia is distressing paroxysmal vasodilation of small arteries in the feet and hands and, less commonly, in the face, ears, or knees; it causes burning pain, increased skin temperature, and redness. […] This rare disorder may be Primary (cause unknown). […] It may also be secondary to Diabetes mellitus, Gout, Hypertension, Lichen sclerosus, Multiple sclerosis, Myeloproliferative disorders (eg, polycythemia vera, thrombocythemia), Rheumatoid arthritis, Spinal cord disorders, Systemic lupus erythematosus, Venous insufficiency. […] Less commonly, the disorder is related to the use of some medications (eg, nifedipine, bromocriptine). […] A rare hereditary form of erythromelalgia starts at birth or during childhood.

• #45 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #46 Erythromelalgia – symptoms, causes and treatment | healthdirect

  https://www.healthdirect.gov.au/erythromelalgia

  Erythromelalgia is a rare skin condition that causes red, painful, warm skin. […] Primary erythromelalgia is caused by inherited genetic differences. […] Secondary erythromelalgia is linked to blood cancers caused by changes to your bone marrow. Occasionally it can be related to: diabetes, infectious diseases, diseases that affect your joints and muscles. […] Symptom flares can be caused by: dehydration not drinking enough fluids, drinking alcohol or eating spicy food, exercise, hot weather, stress, wearing tight clothes and shoes. […] Because the cause of primary erythromelalgia is not well understood, it can’t be prevented. […] Treating the cause of secondary erythromelalgia can help stop the symptoms.

• #47 Erythromelalgia – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/peripheral-arterial-disorders/erythromelalgia

  Erythromelalgia is distressing paroxysmal vasodilation of small arteries in the feet and hands and, less commonly, in the face, ears, or knees; it causes burning pain, increased skin temperature, and redness. […] This rare disorder may be Primary (cause unknown). […] It may also be secondary to Diabetes mellitus, Gout, Hypertension, Lichen sclerosus, Multiple sclerosis, Myeloproliferative disorders (eg, polycythemia vera, thrombocythemia), Rheumatoid arthritis, Spinal cord disorders, Systemic lupus erythematosus, Venous insufficiency. […] Less commonly, the disorder is related to the use of some medications (eg, nifedipine, bromocriptine). […] A rare hereditary form of erythromelalgia starts at birth or during childhood.

• #48 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 is a functional peripheral arterial disease. Usually, the cause of erythromelalgia is unknown. […] A rare hereditary form of erythromelalgia starts at birth or during childhood. Less commonly, the disorder is related to the use of some medications, such as nifedipine (a medication used to treat high blood pressure) or bromocriptine (a medication used to treat Parkinson disease and other disorders). Erythromelalgia also occurs sometimes in people who have certain blood disorders (myeloproliferative disorders), diabetes mellitus, gout, high blood pressure, lichen sclerosus, multiple sclerosis, rheumatoid arthritis, spinal cord disorders, systemic lupus erythematosus (lupus), or venous insufficiency. Erythromelalgia usually develops 2 to 3 years before another underlying disorder is diagnosed. […] 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 present.

• #49 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #50 Erythromelalgia | Maryville, IL | Foot Health Centers

  https://www.foothealthcenters.net/services/foot-ailments/vascular-and-nerve-issues/erythromelalgia/

  If your feet become inflamed or have a burning sensation triggered by warmth, the culprit may be erthromelalgia. […] Erythromelalgia may occur as a primary or secondary disorder. […] The primary form of erythromelalgia is not associated with another condition, and the cause is usually unknown. In rare cases, the disorder is inherited, and symptoms present themselves during childhood. […] Secondary erythromelalgia is associated with an underlying condition. Myeloproliferative (bone marrow) disorders such as polycythemia vera or thrombocythemia are the most common causes of secondary erythromelalgia. However, erythromelalgia often occurs two or three years before a myeloproliferative disorder can be diagnosed. […] Secondary erythromelalgia can also be associated with other conditions including: Certain medications (i.e. bromocriptine, which is used to treat Parkinson’s disease). Diabetes mellitus. Gout. Hypertension. Lead poisoning. Lichen sclerosus. Multiple sclerosis. Rheumatoid arthritis (RA). Spinal cord disorders. Systemic lupus erythematosus (SLE). Venous insufficiency.

• #51 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Primary erythromelalgia may be idiopathic or genetic in origin. Early-onset erythromelalgia can show a familial occurrence. Adult-onset erythromelalgia is idiopathic in the majority of cases, although an inherited case has been reported. […] Hereditary erythromelalgia is an autosomal dominant disorder caused by gain-of-function mutations in SCN9A, which encodes a voltage-gated sodium channel, subtype Nav1.7. More than 20 SCN9A mutations have been reported in patients with primary erythromelalgia. […] Causes of secondary erythromelalgia include: Myeloproliferative disorders, Infection (eg, HIV, poxvirus), Medications, Mercury poisoning, Mushroom poisoning. […] Most reported cases of secondary erythromelalgia are due to myeloproliferative disorders with thrombocytosis (most commonly polycythemia vera or essential thrombocytosis).

• #52 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #53 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #54 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Primary erythromelalgia may be idiopathic or genetic in origin. Early-onset erythromelalgia can show a familial occurrence. Adult-onset erythromelalgia is idiopathic in the majority of cases, although an inherited case has been reported. […] Hereditary erythromelalgia is an autosomal dominant disorder caused by gain-of-function mutations in SCN9A, which encodes a voltage-gated sodium channel, subtype Nav1.7. More than 20 SCN9A mutations have been reported in patients with primary erythromelalgia. […] Causes of secondary erythromelalgia include: Myeloproliferative disorders, Infection (eg, HIV, poxvirus), Medications, Mercury poisoning, Mushroom poisoning. […] Most reported cases of secondary erythromelalgia are due to myeloproliferative disorders with thrombocytosis (most commonly polycythemia vera or essential thrombocytosis).

• #55 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #56 Erythromelalgia – Wikipedia

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

  What causes epidemic erythromelalgia in southern China remains unknown although several erythromelalgia-associated poxviruses were isolated from throat swabs of several patients at different counties and from two different seasons. […] The finding of the specific antibody conversion to ATIs of ERPV in patients’ paired sera strengthens the evidence for a possible aetiological role of human ERPV in epidemic erythromelalgia.

• #57 Erythromelalgia – FindZebra

  https://www.findzebra.com/details/qqYkwQB-erythromelalgia?q=

  Several medications, including verapamil and nifedipine, as well as ergot derivatives such as bromocriptine and pergolide, have been associated with medication-induced erythromelalgia. […] The consumption of two species of related fungi, Clitocybe acromelalga from Japan, and Clitocybe amoenolens from France, has led to several cases of mushroom-induced erythromelalgia which lasted from 8 days to 5 months. […] An epidemic form of this syndrome occurs in secondary students in rural China. In 1987 a virus – erythromelalgia-associated poxvirus – was reported to have been recovered from throat swabs from such an outbreak. […] There are 10 known mutations in the voltage-gated sodium channel -subunit NaV1.7 encoding gene, SCN9A. […] This channel is expressed primarily in nociceptors of the dorsal root ganglion and the sympathetic ganglion neurons.

• #58 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #59 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #60 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #61 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Primary erythromelalgia may be idiopathic or genetic in origin. Early-onset erythromelalgia can show a familial occurrence. Adult-onset erythromelalgia is idiopathic in the majority of cases, although an inherited case has been reported. […] Hereditary erythromelalgia is an autosomal dominant disorder caused by gain-of-function mutations in SCN9A, which encodes a voltage-gated sodium channel, subtype Nav1.7. More than 20 SCN9A mutations have been reported in patients with primary erythromelalgia. […] Causes of secondary erythromelalgia include: Myeloproliferative disorders, Infection (eg, HIV, poxvirus), Medications, Mercury poisoning, Mushroom poisoning. […] Most reported cases of secondary erythromelalgia are due to myeloproliferative disorders with thrombocytosis (most commonly polycythemia vera or essential thrombocytosis).

• #62 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #63 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #64 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #65 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Medications implicated in erythromelalgia include: Bromocriptine, Nifedipine, felodipine, and nicardipine, Epoprostenol, Topical isopropanol, Alemtuzumab, Osimertinib, Fumaric acid, Simvastatin, Eltrombopag, Cyclosporine, COVID-19 vaccines, Pergolide (withdrawn from the US market in 2007). […] Other disorders with a reported association with erythromelalgia include: Systemic lupus erythematosus (SLE), Diabetes mellitus, Venous insufficiency, Astrocytoma, Rheumatoid arthritis, Gout. […] Whether the above associations are coincidental is not clear.

• #66 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #67 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Medications implicated in erythromelalgia include: Bromocriptine, Nifedipine, felodipine, and nicardipine, Epoprostenol, Topical isopropanol, Alemtuzumab, Osimertinib, Fumaric acid, Simvastatin, Eltrombopag, Cyclosporine, COVID-19 vaccines, Pergolide (withdrawn from the US market in 2007). […] Other disorders with a reported association with erythromelalgia include: Systemic lupus erythematosus (SLE), Diabetes mellitus, Venous insufficiency, Astrocytoma, Rheumatoid arthritis, Gout. […] Whether the above associations are coincidental is not clear.

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

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

  Erythromelalgia is a rare skin condition that affects the feet, hands, arms, and legs. […] The exact causes of erythromelalgia aren’t always known. In most cases of erythromelalgia, no cause is pinpointed. But there are a few known causes. […] Erythromelalgia can be a side effect of certain medications, like: bromocriptine, calcium channel blockers, cyclosporine, iodinated contrast agents, norephedrine, topical isopropanol, rosuvastatin. […] Additionally, it can sometimes be the result of another medical condition. Conditions that may cause erythromelalgia include: multiple sclerosis (MS), peripheral neuropathy and other types of nerve damage, lupus, rheumatoid arthritis, or another autoimmune condition, polycythemia vera and conditions that impact your blood. […] Genetics can also play a role. According to an older 2007 review, a mutation on the SCN9A gene that causes erythromelalgia. This gene mutation can run in families and be passed from one generation to the next. […] Erythromelalgia flare-ups are triggered by exposure to rising temperatures, like when you exercise or are in very warm climates. The condition can occur on its own or be caused by another condition.

• #69 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 is a functional peripheral arterial disease. Usually, the cause of erythromelalgia is unknown. […] A rare hereditary form of erythromelalgia starts at birth or during childhood. Less commonly, the disorder is related to the use of some medications, such as nifedipine (a medication used to treat high blood pressure) or bromocriptine (a medication used to treat Parkinson disease and other disorders). Erythromelalgia also occurs sometimes in people who have certain blood disorders (myeloproliferative disorders), diabetes mellitus, gout, high blood pressure, lichen sclerosus, multiple sclerosis, rheumatoid arthritis, spinal cord disorders, systemic lupus erythematosus (lupus), or venous insufficiency. Erythromelalgia usually develops 2 to 3 years before another underlying disorder is diagnosed. […] 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 present.

• #70 Erythromelalgia | Maryville, IL | Foot Health Centers

  https://www.foothealthcenters.net/services/foot-ailments/vascular-and-nerve-issues/erythromelalgia/

  If your feet become inflamed or have a burning sensation triggered by warmth, the culprit may be erthromelalgia. […] Erythromelalgia may occur as a primary or secondary disorder. […] The primary form of erythromelalgia is not associated with another condition, and the cause is usually unknown. In rare cases, the disorder is inherited, and symptoms present themselves during childhood. […] Secondary erythromelalgia is associated with an underlying condition. Myeloproliferative (bone marrow) disorders such as polycythemia vera or thrombocythemia are the most common causes of secondary erythromelalgia. However, erythromelalgia often occurs two or three years before a myeloproliferative disorder can be diagnosed. […] Secondary erythromelalgia can also be associated with other conditions including: Certain medications (i.e. bromocriptine, which is used to treat Parkinson’s disease). Diabetes mellitus. Gout. Hypertension. Lead poisoning. Lichen sclerosus. Multiple sclerosis. Rheumatoid arthritis (RA). Spinal cord disorders. Systemic lupus erythematosus (SLE). Venous insufficiency.

• #71 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Medications implicated in erythromelalgia include: Bromocriptine, Nifedipine, felodipine, and nicardipine, Epoprostenol, Topical isopropanol, Alemtuzumab, Osimertinib, Fumaric acid, Simvastatin, Eltrombopag, Cyclosporine, COVID-19 vaccines, Pergolide (withdrawn from the US market in 2007). […] Other disorders with a reported association with erythromelalgia include: Systemic lupus erythematosus (SLE), Diabetes mellitus, Venous insufficiency, Astrocytoma, Rheumatoid arthritis, Gout. […] Whether the above associations are coincidental is not clear.

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

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

  Erythromelalgia is a rare skin condition that affects the feet, hands, arms, and legs. […] The exact causes of erythromelalgia aren’t always known. In most cases of erythromelalgia, no cause is pinpointed. But there are a few known causes. […] Erythromelalgia can be a side effect of certain medications, like: bromocriptine, calcium channel blockers, cyclosporine, iodinated contrast agents, norephedrine, topical isopropanol, rosuvastatin. […] Additionally, it can sometimes be the result of another medical condition. Conditions that may cause erythromelalgia include: multiple sclerosis (MS), peripheral neuropathy and other types of nerve damage, lupus, rheumatoid arthritis, or another autoimmune condition, polycythemia vera and conditions that impact your blood. […] Genetics can also play a role. According to an older 2007 review, a mutation on the SCN9A gene that causes erythromelalgia. This gene mutation can run in families and be passed from one generation to the next. […] Erythromelalgia flare-ups are triggered by exposure to rising temperatures, like when you exercise or are in very warm climates. The condition can occur on its own or be caused by another condition.

• #73 Erythromelalgia: Practice Essentials, Background, Pathophysiology

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

  Medications implicated in erythromelalgia include: Bromocriptine, Nifedipine, felodipine, and nicardipine, Epoprostenol, Topical isopropanol, Alemtuzumab, Osimertinib, Fumaric acid, Simvastatin, Eltrombopag, Cyclosporine, COVID-19 vaccines, Pergolide (withdrawn from the US market in 2007). […] Other disorders with a reported association with erythromelalgia include: Systemic lupus erythematosus (SLE), Diabetes mellitus, Venous insufficiency, Astrocytoma, Rheumatoid arthritis, Gout. […] Whether the above associations are coincidental is not clear.

• #74 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #75 Erythromelalgia: A Very Rare Condition

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

  People with autoimmune diseases, such as diabetes, lupus, Sjgrens syndrome, or vascular disorders, are believed to have a higher risk of EM. Other health conditions may trigger it, including myeloproliferative diseases. Some neurological diseases increase risk, including multiple sclerosis. […] Heavy metal poisoning has been linked to EM. This is likely the result of toxic amounts of these substances, such as mercury and arsenic, entering soft tissues in the body. […] Some medications, including ergot derivatives, have been linked to EM. […] Diet has also been linked to EM. Spicy foods and overconsumption of alcohol, for example, are commonly reported triggers for EM flares. […] Aside from genetics, many causes and risk factors are speculative, and researchers believe that in the majority of people, the cause of EM is unknown.

• #76 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #77 Erythromelalgia – FindZebra

  https://www.findzebra.com/details/qqYkwQB-erythromelalgia?q=

  Several medications, including verapamil and nifedipine, as well as ergot derivatives such as bromocriptine and pergolide, have been associated with medication-induced erythromelalgia. […] The consumption of two species of related fungi, Clitocybe acromelalga from Japan, and Clitocybe amoenolens from France, has led to several cases of mushroom-induced erythromelalgia which lasted from 8 days to 5 months. […] An epidemic form of this syndrome occurs in secondary students in rural China. In 1987 a virus – erythromelalgia-associated poxvirus – was reported to have been recovered from throat swabs from such an outbreak. […] There are 10 known mutations in the voltage-gated sodium channel -subunit NaV1.7 encoding gene, SCN9A. […] This channel is expressed primarily in nociceptors of the dorsal root ganglion and the sympathetic ganglion neurons.

• #78 Erythromelalgia – StatPearls – NCBI Bookshelf

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

  Erythromelalgia in thrombocythemia occurs in association with essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia may be idiopathic or inherited. The inherited form of erythromelalgia is an autosomal dominant neuropathy caused by a gain-of-function mutation in the SCN9A, SCN10A, and SCN11A gene, which encodes the alpha subunit of the voltage-gated NaV 1.7, NaV 1.8, and NaV 1.9 sodium channel, respectively. […] Secondary erythromelalgia has been attributed to a number of different medical conditions. The most prevalent being myeloproliferative disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis. Other underlying causes include infectious agents (HIV, influenza, syphilis, and poxvirus), autoimmune diseases (systemic lupus erythematosus and rheumatoid arthritis), diabetes mellitus type 1 and 2, solid tumors (astrocytoma, colon, and breast cancer), medications (bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, simvastatin), gout, multiple sclerosis, hypertension, venous insufficiency, pernicious anemia, thrombotic thrombocytopenic purpura, mushroom intoxication, and mercury poisoning.

• #79 Erythromelalgia | Thoracic Key

  https://thoracickey.com/erythromelalgia/

  Erythromelalgia is a rare condition of the extremities characterized by the triad of redness, warmth, and pain. […] It has been argued that William Harvey could have had erythromelalgia, not gout. […] Erythromelalgia has been reported in association with myeloproliferative diseases, blood disorders, drugs, infectious diseases, food ingestion (mushrooms), neoplasms, connective tissue disease, physiological conditions (pregnancy), and neuropathies. […] The relationship of many underlying disorders to erythromelalgia is sometimes unclear, and the disorder may be a coincidental comorbidity rather than an underlying disease. […] Among the reported series, the association with myeloproliferative disease seems most constant. […] Evidence of underlying myeloproliferative disease should be sought at diagnosis and subsequently. Erythromelalgia can herald the onset of underlying myeloproliferative disease.

• #80 Erythromelalgia – BAD Patient Hub

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

  Erythromelalgia is thought to be due to problems with nerves running from the brain to blood vessels in the affected area. As a result, there is an increase in blood flow to the affected area which leads to pain, swelling and redness. […] Primary erythromelalgia is caused by a change in your pain nerves due to a change in one of your genes. […] The rarer secondary form comes on later in life usually between the age of 40 60 years and is usually linked to another illness. […] Tests might be done to rule out other rare causes, such as medications, diabetes, lupus, HIV, bone marrow disorders, and pregnancy. […] In very rare cases, there may be other cause, including leukaemia. […] Primary erythromelalgia can run in families, so there is a one in two chance of an affected person passing this onto each child they have. However, sometimes a family history of erythromelalgia is not found (this is called sporadic mutation).

• #81 ABSTRACT

  https://www.ejcrim.com/index.php/EJCRIM/article/download/1852/2293?inline=1

  A 61-year-old woman with a history of primary aldosteronism caused by unilateral hyperplasia of the adrenal gland presented with a 2-week history of redness and severe pain in the right thumb and thenar regions. […] Hence, we diagnosed the patient with erythromelalgia caused by polycythemia vera. […] Polycythemia can cause erythromelalgia, which should be treated with aspirin. […] Primary aldosteronism causes secondary erythropoiesis through activation of the renin-aldosterone system, but the mechanism is not clear. […] Erythropoiesis may be promoted by concurrent primary aldosteronism and polycythemia vera, resulting in secondary erythromelalgia. […] Erythromelalgia causes redness and pain in the upper and lower limbs. It is classified as primary (caused by gene mutations) and secondary erythromelalgia (associated with underlying factors, such as myeloproliferative causes including polycythemia, drug use, infection and collagen disease).

• #82 Erythromelalgia | 5-Minute Clinical Consult

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

  Primary EM has two subcategories: idiopathic and inherited. […] Secondary EM is acquired and associated with systemic diseases or other external causes. […] Two major etiologic theories, vascular and neurologic, which may not be mutually exclusive: Microvascular shunting: Increased thermoregulatory perfusion with inadequate capillary perfusion in the affected skin causes tissue hypoxia, leading to reactive arteriole dilation and hyperemia. […] Neurologic: Primary hereditary EM has a component of small fiber involving cutaneous sympathetic and sensory fibers. A defect in NaV1.7 sodium channels causes nerve hyperexcitability and pain. This hyperexcitability may also cause a secondary vascular maldistribution and induce hypoxia. […] Most patients with EM have either the idiopathic form or the secondary form, neither of which have a known genetic component.

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

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

  Erythromelalgia is an infrequent episodic acrosyndrome affecting mainly both lower limbs symmetrically with the classic triad of erythema, warmth and burning pain. […] Primary erythromelalgia is an autosomal dominant inherited disorder, while secondary is associated with myeloproliferative diseases, among others. […] In its etiopathogenesis, there are neural and vascular abnormalities that can be combined. […] Primary EM is an autosomal dominant inherited disorder encoded by OMIN (Online Mendelian Inheritance in Man) as #133020. It is associated with an alteration on the subunit protein of the sodium channel type 9 (SCN9A), affecting the Nav1.7 channel that is expressed mainly in dorsal root ganglia and the sympathetic ganglia neurons. […] Another inherited form, not related to SCN9A, has been recently reported.

• #84 Erythromelalgia – Wikipedia

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

  There was a study done in 2013 where two Vietnamese patients were diagnosed with primary erythromelalgia. […] A genetic analysis was performed where scientists observed three missense mutations, one familial and two sporadic mutations. […] Primary erythromelalgia is caused by mutation of the voltage-gated sodium channel -subunit gene SCN9A. […] In 2004 erythromelalgia became the first human disorder in which it has been possible to associate an ion channel mutation with chronic neuropathic pain, when its link to the SCN9A gene was initially published. […] The neuropathological symptoms of primary erythromelalgia arise from hyperexcitability of C-fibers in the dorsal root ganglion. […] This hyperexcitability results in the severe burning pain experienced by patients. […] In both cases, these changes in excitability are typically due to mutation of the sodium channel NaV1.7.

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

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

  Secondary EM could precede the underlying disease in at least two years and could be present in children as well as in adults. Myeloproliferative diseases, paraneoplasias, autoimmune diseases, toxins and infections are among the associated disorders. […] The risk of secondary EM associated with myeloproliferative disease is 3% to 65%. […] Some authors consider myeloproliferative disorders and essential thrombocytopenia to be responsible for 20% of the cases in secondary EM. […] Previous studies propose that occlusion of the arterial microcirculation could be triggered by platelet activation and aggregation in these patients. […] The altered electric excitability of neurons can increase pain. In primary familiar and sporadic EM cases there is an alteration of the alpha subunit of the Nav1.7 sodium channel due genetic mutation.

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

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

  Secondary EM could precede the underlying disease in at least two years and could be present in children as well as in adults. Myeloproliferative diseases, paraneoplasias, autoimmune diseases, toxins and infections are among the associated disorders. […] The risk of secondary EM associated with myeloproliferative disease is 3% to 65%. […] Some authors consider myeloproliferative disorders and essential thrombocytopenia to be responsible for 20% of the cases in secondary EM. […] Previous studies propose that occlusion of the arterial microcirculation could be triggered by platelet activation and aggregation in these patients. […] The altered electric excitability of neurons can increase pain. In primary familiar and sporadic EM cases there is an alteration of the alpha subunit of the Nav1.7 sodium channel due genetic mutation.

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

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

  The risk of secondary EM associated with myeloproliferative disease is 3% to 65%. Some authors consider myeloproliferative disorders and essential thrombocytopenia to be responsible for 20% of the cases in secondary EM. […] The primary afferents contribute to this multifaceted inflammatory process, releasing neuropeptides that take part in a sort of oil spot nociceptor sensitization. […] The activation and modification of the receptor sensitivity are produced by changes on neural membrane of the sodium, potassium and calcium conductance. […] The sodium channels controlled by voltage Nav are key determinants of the nociceptor excitability and play a fundamental role in pain transmission. […] The altered electric excitability of neurons can increase pain. In primary familiar and sporadic EM cases there is an alteration of the alpha subunit of the Nav1.7 sodium channel due genetic mutation.

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

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

  This mutation produces hyperexcitability of the sensorial peripheral and sympathetic neurons, decreasing the threshold and thus increasing the discharge frequency of the Nav 1.7 mutant of neural ganglions of dorsal roots. […] The activation of the ionic channels produces the activation of the action potential and also the release of inflammation mediators. […] The sodium channels controlled by voltage Nav are key determinants of the nociceptor excitability and play a fundamental role in pain transmission. […] The primary afferents contribute to this multifaceted inflammatory process, releasing neuropeptides that take part in a sort of „oil spot nociceptor sensitization.” […] The dysfunction of the microcirculation that causes the symptoms. […] The increased perfusion of the affected limb is paradoxically associated with tissue ischemia due to an abnormal distribution of the microvascular flow through arteriovenous pre-capillary shunts.

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

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

  The risk of secondary EM associated with myeloproliferative disease is 3% to 65%. Some authors consider myeloproliferative disorders and essential thrombocytopenia to be responsible for 20% of the cases in secondary EM. […] The primary afferents contribute to this multifaceted inflammatory process, releasing neuropeptides that take part in a sort of oil spot nociceptor sensitization. […] The activation and modification of the receptor sensitivity are produced by changes on neural membrane of the sodium, potassium and calcium conductance. […] The sodium channels controlled by voltage Nav are key determinants of the nociceptor excitability and play a fundamental role in pain transmission. […] The altered electric excitability of neurons can increase pain. In primary familiar and sporadic EM cases there is an alteration of the alpha subunit of the Nav1.7 sodium channel due genetic mutation.

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

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

  The opening of arteriovenous anastomoses with the consequent flow increase leads to a decrease of the capillary circulation, diminishing the oxygenated blood and producing hypoxia and tissue pain. […] The diagnosis of small fibers neuropathy is observed in most of these patients. […] The impaired vascular distribution leading to hypoxia could trigger the neuropathy. […] The presence of erythema pernio and in second place, livedo reticularis, is frequent in patients with acrocyanosis, erythromelalgia and less in cases of Raynaud`s disease. […] The diabetic microangiopathy and neuropathy could add up to the EM with an overlapping of signs and symptoms generating difficulties in the diagnosis and also complicating the disorder.

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

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

  The pain due to small fibers and C-nociceptor produces typical symptoms such as aching feet, burning pain and puncture or a cutting knife sensation. […] The increased perfusion of the affected limb is paradoxically associated with tissue ischemia due to an abnormal distribution of the microvascular flow through arteriovenous pre-capillary shunts. […] The impaired vascular distribution leading to hypoxia could trigger the neuropathy. […] The altered activity of the sodium channel Nav1.7 is present in both sympathetic and sensory nerves.

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

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

  Secondary EM could precede the underlying disease in at least two years and could be present in children as well as in adults. Myeloproliferative diseases, paraneoplasias, autoimmune diseases, toxins and infections are among the associated disorders. […] The risk of secondary EM associated with myeloproliferative disease is 3% to 65%. […] Some authors consider myeloproliferative disorders and essential thrombocytopenia to be responsible for 20% of the cases in secondary EM. […] Previous studies propose that occlusion of the arterial microcirculation could be triggered by platelet activation and aggregation in these patients. […] The altered electric excitability of neurons can increase pain. In primary familiar and sporadic EM cases there is an alteration of the alpha subunit of the Nav1.7 sodium channel due genetic mutation.

• #93 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia (ur-i-thruh-muh-lal-jah) is a rare condition in which excess blood flow causes periodic symptom flares. Its a chronic condition, meaning that it stays with you for life. Erythromelalgia symptoms, which often include burning pain, worsen over time. […] Primary erythromelalgia is isolated and not due to an underlying disease. […] Idiopathic erythromelalgia is the most common type and occurs for unknown reasons. […] Inherited erythromelalgia is due to gene mutations. The mutations, which affect pain signals, can be passed down in families. […] Secondary erythromelalgia occurs when there is an underlying blood, neurological or immunologic condition. […] Flares usually occur when you experience a sudden increase in body temperature. […] 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.

• #94 Erythromelalgia

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

  It’s often not clear what causes erythromelalgia. […] Sometimes it’s caused by another condition or a faulty gene inherited from one of your parents. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] This can be caused by: exercise, wearing warm or tight clothes and shoes, warm temperatures (such as during hot weather), stress, drinking alcohol or eating spicy food, not drinking enough fluids (dehydration).

• #95 Erythromelalgia

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

  It’s often not clear what causes erythromelalgia. […] Sometimes it’s caused by another condition or a faulty gene inherited from one of your parents. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] This can be caused by: exercise, wearing warm or tight clothes and shoes, warm temperatures (such as during hot weather), stress, drinking alcohol or eating spicy food, not drinking enough fluids (dehydration).

• #96 Erythromelalgia – symptoms, causes and treatment | healthdirect

  https://www.healthdirect.gov.au/erythromelalgia

  Erythromelalgia is a rare skin condition that causes red, painful, warm skin. […] Primary erythromelalgia is caused by inherited genetic differences. […] Secondary erythromelalgia is linked to blood cancers caused by changes to your bone marrow. Occasionally it can be related to: diabetes, infectious diseases, diseases that affect your joints and muscles. […] Symptom flares can be caused by: dehydration not drinking enough fluids, drinking alcohol or eating spicy food, exercise, hot weather, stress, wearing tight clothes and shoes. […] Because the cause of primary erythromelalgia is not well understood, it can’t be prevented. […] Treating the cause of secondary erythromelalgia can help stop the symptoms.

• #97 Erythromelalgia

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

  It’s often not clear what causes erythromelalgia. […] Sometimes it’s caused by another condition or a faulty gene inherited from one of your parents. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] This can be caused by: exercise, wearing warm or tight clothes and shoes, warm temperatures (such as during hot weather), stress, drinking alcohol or eating spicy food, not drinking enough fluids (dehydration).

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

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

  Erythromelalgia is a rare skin condition that affects the feet, hands, arms, and legs. […] The exact causes of erythromelalgia aren’t always known. In most cases of erythromelalgia, no cause is pinpointed. But there are a few known causes. […] Erythromelalgia can be a side effect of certain medications, like: bromocriptine, calcium channel blockers, cyclosporine, iodinated contrast agents, norephedrine, topical isopropanol, rosuvastatin. […] Additionally, it can sometimes be the result of another medical condition. Conditions that may cause erythromelalgia include: multiple sclerosis (MS), peripheral neuropathy and other types of nerve damage, lupus, rheumatoid arthritis, or another autoimmune condition, polycythemia vera and conditions that impact your blood. […] Genetics can also play a role. According to an older 2007 review, a mutation on the SCN9A gene that causes erythromelalgia. This gene mutation can run in families and be passed from one generation to the next. […] Erythromelalgia flare-ups are triggered by exposure to rising temperatures, like when you exercise or are in very warm climates. The condition can occur on its own or be caused by another condition.

• #99 Erythromelalgia

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

  It’s often not clear what causes erythromelalgia. […] Sometimes it’s caused by another condition or a faulty gene inherited from one of your parents. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] This can be caused by: exercise, wearing warm or tight clothes and shoes, warm temperatures (such as during hot weather), stress, drinking alcohol or eating spicy food, not drinking enough fluids (dehydration).

• #100 Erythromelalgia – symptoms, causes and treatment | healthdirect

  https://www.healthdirect.gov.au/erythromelalgia

  Erythromelalgia is a rare skin condition that causes red, painful, warm skin. […] Primary erythromelalgia is caused by inherited genetic differences. […] Secondary erythromelalgia is linked to blood cancers caused by changes to your bone marrow. Occasionally it can be related to: diabetes, infectious diseases, diseases that affect your joints and muscles. […] Symptom flares can be caused by: dehydration not drinking enough fluids, drinking alcohol or eating spicy food, exercise, hot weather, stress, wearing tight clothes and shoes. […] Because the cause of primary erythromelalgia is not well understood, it can’t be prevented. […] Treating the cause of secondary erythromelalgia can help stop the symptoms.

• #101 Erythromelalgia

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

  It’s often not clear what causes erythromelalgia. […] Sometimes it’s caused by another condition or a faulty gene inherited from one of your parents. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] This can be caused by: exercise, wearing warm or tight clothes and shoes, warm temperatures (such as during hot weather), stress, drinking alcohol or eating spicy food, not drinking enough fluids (dehydration).

• #102 Erythromelalgia – symptoms, causes and treatment | healthdirect

  https://www.healthdirect.gov.au/erythromelalgia

  Erythromelalgia is a rare skin condition that causes red, painful, warm skin. […] Primary erythromelalgia is caused by inherited genetic differences. […] Secondary erythromelalgia is linked to blood cancers caused by changes to your bone marrow. Occasionally it can be related to: diabetes, infectious diseases, diseases that affect your joints and muscles. […] Symptom flares can be caused by: dehydration not drinking enough fluids, drinking alcohol or eating spicy food, exercise, hot weather, stress, wearing tight clothes and shoes. […] Because the cause of primary erythromelalgia is not well understood, it can’t be prevented. […] Treating the cause of secondary erythromelalgia can help stop the symptoms.

• #103 Erythromelalgia

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

  It’s often not clear what causes erythromelalgia. […] Sometimes it’s caused by another condition or a faulty gene inherited from one of your parents. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] This can be caused by: exercise, wearing warm or tight clothes and shoes, warm temperatures (such as during hot weather), stress, drinking alcohol or eating spicy food, not drinking enough fluids (dehydration).

• #104 Erythomelalgia | SRUK

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

  What causes Erythromelalgia? For most people with the condition, the cause is unknown but some underlying cause may be found such as a medical condition or faulty gene. […] Underlying causes include: Having abnormally high levels of blood cells […] Damage to the peripheral nervous system […] Multiple Sclerosis […] An autoimmune condition […] What causes flare-ups? Warm temperatures seem to be the most frequent trigger, something which is especially problematic for those with Raynaud’s. Others have found that spices and alcohol can also make the symptoms worse. […] Primary – Primary EM is not caused by an underlying disease. It includes both inherited and idiopathic EM. […] Secondary – Cases of EM that are caused by another disease or condition are called secondary EM. The most common causes of secondary EM are: Essential thrombocythemia […] Polycythemia vera […] Thrombocytopenia […] Peripheral neuropathy […] Autoimmune diseases […] Nerve damage due to injuries or other conditions […] In some cases, when the underlying condition is treated, EM symptoms will improve or resolve completely.

• #105 Erythromelalgia

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

  It’s often not clear what causes erythromelalgia. […] Sometimes it’s caused by another condition or a faulty gene inherited from one of your parents. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] This can be caused by: exercise, wearing warm or tight clothes and shoes, warm temperatures (such as during hot weather), stress, drinking alcohol or eating spicy food, not drinking enough fluids (dehydration).

• #106 Erythomelalgia | SRUK

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

  What causes Erythromelalgia? For most people with the condition, the cause is unknown but some underlying cause may be found such as a medical condition or faulty gene. […] Underlying causes include: Having abnormally high levels of blood cells […] Damage to the peripheral nervous system […] Multiple Sclerosis […] An autoimmune condition […] What causes flare-ups? Warm temperatures seem to be the most frequent trigger, something which is especially problematic for those with Raynaud’s. Others have found that spices and alcohol can also make the symptoms worse. […] Primary – Primary EM is not caused by an underlying disease. It includes both inherited and idiopathic EM. […] Secondary – Cases of EM that are caused by another disease or condition are called secondary EM. The most common causes of secondary EM are: Essential thrombocythemia […] Polycythemia vera […] Thrombocytopenia […] Peripheral neuropathy […] Autoimmune diseases […] Nerve damage due to injuries or other conditions […] In some cases, when the underlying condition is treated, EM symptoms will improve or resolve completely.

• #107 Erythromelalgia

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

  It’s often not clear what causes erythromelalgia. […] Sometimes it’s caused by another condition or a faulty gene inherited from one of your parents. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] This can be caused by: exercise, wearing warm or tight clothes and shoes, warm temperatures (such as during hot weather), stress, drinking alcohol or eating spicy food, not drinking enough fluids (dehydration).

• #108 Erythromelalgia – symptoms, causes and treatment | healthdirect

  https://www.healthdirect.gov.au/erythromelalgia

  Erythromelalgia is a rare skin condition that causes red, painful, warm skin. […] Primary erythromelalgia is caused by inherited genetic differences. […] Secondary erythromelalgia is linked to blood cancers caused by changes to your bone marrow. Occasionally it can be related to: diabetes, infectious diseases, diseases that affect your joints and muscles. […] Symptom flares can be caused by: dehydration not drinking enough fluids, drinking alcohol or eating spicy food, exercise, hot weather, stress, wearing tight clothes and shoes. […] Because the cause of primary erythromelalgia is not well understood, it can’t be prevented. […] Treating the cause of secondary erythromelalgia can help stop the symptoms.

• #109 Erythromelalgia: Causes, Symptoms, and Treatment

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

  Erythromelalgia is a rare and often debilitating condition characterized by episodes of intense burning pain, warmth, and redness of the extremities. […] The exact cause of erythromelalgia is not fully understood, but several factors are believed to play a role: […] Genetic Mutations: Mutations in the SCN9A gene can lead to primary erythromelalgia. […] Vascular Abnormalities: Abnormalities in blood vessel function and blood flow are considered contributing factors. […] Nerve Damage: Peripheral nerve dysfunction or damage may be involved. […] Inflammatory Processes: Chronic inflammation might exacerbate the condition. […] Erythromelalgia is caused by blood vessel abnormalities that result in excessive blood flow to the extremities, leading to redness and burning pain.

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

  https://qwarkhealth.com/conditions/erythromelalgia

  Erythromelalgia is a rare neurovascular peripheral disorder that causes intermittent or continuous episodes of bilateral redness, warmth and burning pain in the extremities. […] It is caused by mutations in genes that regulate the neurovascular response to heat, and may be hereditary or acquired. […] The exact cause of erythromelalgia is still unknown. However, it is believed that it is caused by the dysfunction of the endothelial cells lining the blood vessels, leading to an increased release of vasodilating agents such as nitric oxide, serotonin and prostaglandin E2, which leads to the dilation of blood vessels and increased blood flow to the affected areas. […] The condition can also be triggered by various factors, including genetic mutations, autoimmune disorders, and exposure to certain medications, toxins and infections. […] Erythromelalgia can be hereditary, caused by genetic mutations.

• #111 Erythromelalgia – MD Searchlight

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

  Erythromelalgia is a condition often linked with blood disorders such as essential thrombocytosis and polycythemia vera. […] Primary erythromelalgia could occur without a known reason (idiopathic) or could be inherited. The inherited type is due to a problematic change in one of the genes SCN9A, SCN10A, or SCN11A. These genes control parts of the channel that allows sodium to enter nerve cells, specifically in a part of the nervous system called the dorsal root ganglion. The problematic change leads to overactivity in pain-sensing nerve fibers, causing them to respond to stimuli that shouldn’t be painful. […] Secondary erythromelalgia is linked with a variety of medical conditions. Most commonly, it coincides with blood disorders, including essential thrombocytosis, polycythemia vera, and myelofibrosis (all conditions that involve an overproduction of blood cells). Other causes can range from infectious diseases such as HIV, flu, syphilis and poxvirus, autoimmune diseases like lupus and rheumatoid arthritis, both type 1 and type 2 diabetes, certain cancers (e.g. brain, colon and breast cancer), gout, multiple sclerosis, high blood pressure, vein problems, a type of anemia called pernicious anemia, a rare blood disorder (thrombotic thrombocytopenic purpura), to intoxication from mushrooms and mercury poisoning. Certain medications like bromocriptine, nifedipine, verapamil, topical isopropanol, pergolide, and simvastatin can also trigger secondary erythromelalgia.

• #112 Erythromelalgia – MD Searchlight

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

  Erythromelalgia can be acquired through blood disorders, such as essential thrombocytosis and polycythemia vera, or it can be inherited due to problematic changes in genes SCN9A, SCN10A, or SCN11A. It can also be linked to a variety of medical conditions, including infectious diseases, autoimmune diseases, certain cancers, gout, multiple sclerosis, high blood pressure, vein problems, anemia, rare blood disorders, and intoxication from mushrooms and mercury poisoning. Certain medications can also trigger erythromelalgia.

• #113 Erythromelalgia: Symptoms, Causes, Treatment

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

  Erythromelalgia (ur-i-thruh-muh-lal-jah) is a rare condition in which excess blood flow causes periodic symptom flares. Its a chronic condition, meaning that it stays with you for life. Erythromelalgia symptoms, which often include burning pain, worsen over time. […] Primary erythromelalgia is isolated and not due to an underlying disease. […] Idiopathic erythromelalgia is the most common type and occurs for unknown reasons. […] Inherited erythromelalgia is due to gene mutations. The mutations, which affect pain signals, can be passed down in families. […] Secondary erythromelalgia occurs when there is an underlying blood, neurological or immunologic condition. […] Flares usually occur when you experience a sudden increase in body temperature. […] 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.

• #114

  https://journals.lww.com/pain/fulltext/2017/05000/burning_pain__axonal_dysfunction_in.17.aspx

  The pathophysiological mechanisms leading to the clinical attacks with increasing temperature in EM raise the importance of investigating their effects on axonal dysfunction. […] The most striking result was the significant increases in SDTC in EMSCN9A+ patients, an indirect measure of the activity of nodal persistent Na+ current with heating, the opposite of controls. […] Nav1.7-mediated disturbances in vascular regulation may underlie the distinct electrophysiological changes observed in myelinated motor and cutaneous afferents in this study, leading to membrane hyperpolarization following nerve ischemia. […] A paradoxical pattern of changes in Na+ channel-dependent parameters with heating was evident in SCN9A mutant patients in comparison to normal controls. […] There is an urgent clinical need to develop more effective Na+ channel blockers for the treatment of neuropathic pain, particularly the selective and potent Nav1.7 channel blockers, with initial results showing promise in EM.

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