986 results match your criteria: "Erythromelalgia"

Essential thrombocytosis (ET) is a hematopoietic stem cell disorder in which the overproduction of platelets without a definable cause occurs. Its incidence is 1-2 per 100,000 population with female preponderance. These myeloproliferative disorders are associated with many dermatological manifestations such as pruritus and erythromelalgia.

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Article Synopsis
  • - Polycythemia vera (PV) is a rare blood disorder affecting around 65,000 Americans, characterized by an increased mass of red blood cells, leading to a higher risk of blood clots, with symptoms such as itching and enlarged spleen.
  • - Diagnosis often involves identifying elevated hemoglobin levels and the presence of a specific JAK2 gene variant, with many patients also experiencing increased platelet and white blood cell counts; survival rates range from 14 to 28 years following diagnosis.
  • - Treatment typically includes therapeutic phlebotomy and low-dose aspirin for thrombosis prevention, with higher-risk patients possibly receiving additional therapies like hydroxyurea or Ruxolitinib for symptom relief; a small percentage may
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Objectives: Hemorrhagic events are a rare but potentially fatal complication in patients with polycythemia vera (PV).

Methods: We analyzed the characteristics of hemorrhagic events in 267 patients with PV.

Results: A median follow-up of 4.

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Article Synopsis
  • - Polycythemia vera (PV) is a serious blood condition characterized by an increase in red blood cells (erythrocytosis) and symptoms like headaches and weakness, often requiring the exclusion of other causes before diagnosis.
  • - A case of a 47-year-old woman highlights the diagnostic process for PV, which included high hemoglobin levels and a positive JAK-2 mutation test, ultimately leading to the diagnosis despite elevated erythropoietin (EPO) levels.
  • - Treatment for PV primarily involves phlebotomy to lower high blood counts, with additional options like low-dose aspirin for high-risk patients and anticoagulation for those experiencing blood clots.
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Interplay of Nav1.8 and Nav1.7 channels drives neuronal hyperexcitability in neuropathic pain.

J Gen Physiol

November 2024

Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT, USA.

Article Synopsis
  • This study investigates how voltage-gated sodium channels Nav1.7 and Nav1.8 work together in dorsal root ganglion (DRG) neurons, particularly focusing on the impact of a mutation in Nav1.7 associated with neuropathic pain known as inherited erythromelalgia (IEM).
  • Researchers found that Nav1.8 significantly increases the likelihood of action potential (AP) generation near the voltage threshold, outperforming Nav1.7 in terms of channel open-probability at -21.9 mV by nine times.
  • Reducing Nav1.8 current by 25-50% can decrease the excitability of DRG neurons with the Nav1.7
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Erythromelalgia is a rare disorder characterized by episodic burning pain with redness and warmth of the extremities. Topical and systemic medications are the mainstay of management. We reviewed the published evidence for using procedural interventions to manage erythromelalgia, including their proposed mechanism of action and possible adverse effects, and included information in this review on epidural infusion, sympathetic ganglion block, sympathectomy, pulsed radiofrequency, spinal cord stimulation, dorsal root ganglion stimulation, brain stimulation, transcranial magnetic stimulation, and botulinum toxin injections.

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Article Synopsis
  • Erythromelalgia is a rare condition that causes severe pain, redness, and hot flashes in the legs.
  • A 40-year-old woman with 15 years of persistent symptoms found relief through Kampo medicine and acupuncture after failing to respond to several conventional treatments.
  • The case suggests that combining traditional therapies like Kampo medicine with acupuncture could enhance the quality of life for those suffering from erythromelalgia, as there is currently no standard treatment available.
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Human pain channelopathies.

Handb Clin Neurol

August 2024

Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom. Electronic address:

Article Synopsis
  • Significant advancements have been made in understanding how nociceptors, pain-sensing neurons, convert damaging stimuli into pain signals, primarily through specific ion channels.
  • Mutations in certain ion channels, like the voltage-gated sodium channel Na1.7, have been tied to inherited pain disorders, with loss-of-function mutations leading to an inability to feel pain and gain-of-function mutations causing various painful conditions.
  • Other ion channels, such as TRPA1, and variants in VGSCs 1.8 and 1.9, have also been associated with pain disorders, highlighting the potential for targeting these channels in developing new pain medications and tailoring existing treatments based on genetic profiles.
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Relapsing-Remitting Immunotherapy Responsive Small-Fiber Neuropathy: Longitudinal Tracking Through 10 Years Including Pregnancies.

Neurol Neuroimmunol Neuroinflamm

September 2024

From the Department of Neurology (A.L.O.), Massachusetts General Hospital and Harvard Medical School; Department of Pathology (Neuropathology) (A.L.O.), Massachusetts General Hospital; Harvard T.H. Chan School of Public Health & Harvard Medical School (J.A.), Boston; Department of Medical Oncology and Hematology (N.D.), University Hospital Zurich; Department of Neurology (E.P.W.-S.), University of Bern; and Department Neruology (Neurozentrum) (E.P.W.-S.), Luzerner Kantonsspital, Switzerland.

Objectives: To expand understanding of the pathogenesis, presentations, and treatment of initially idiopathic small fiber polyneuropathy (SFN).

Methods: We longitudinally readministered validated metrics to track disease course and treatment responses in a previously healthy woman with acute, postinfectious, skin biopsy-confirmed, idiopathic SFN.

Results: During 5 years, viral respiratory infections triggered 3 separated episodes of acute, disabling burning hand, foot, and face pain (erythromelalgia).

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Background: Red Ear Syndrome is a burning sensation and erythema of the ear, associated with a various number of disorders including migraine, trigeminal neuralgia, autoimmune disorders etc. Theories for RES pathophysiology have developed from current understandings of comorbid conditions. Characterizing the underlying mechanism of RES is crucial for defining effective treatments.

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Stellate ganglion block beyond chronic pain: A literature review on its application in painful and non-painful conditions.

J Anaesthesiol Clin Pharmacol

March 2023

Department of Anesthesia and Perioperative Medicine, Western University, London, Ontario, Canada.

Cervical sympathetic or stellate ganglion blocks (SGBs) have been commonly used in the treatment of painful conditions like complex regional pain syndrome (CRPS). However, there is literature to suggest its utility in managing non-painful conditions as well. The focus of this literature review is to provide an overview of indications for SGB for painful and non-painful conditions.

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Painful Raynaud's mimics.

Best Pract Res Clin Rheumatol

March 2024

Department of Internal Medicine, Division of Rheumatology, University of Michigan, 300 North Ingalls Building - Rm 7C27, Ann Arbor, MI, 48109, USA. Electronic address:

Article Synopsis
  • Raynaud's syndrome is commonly associated with autoimmune diseases and requires proper diagnosis to differentiate it from other similar conditions.
  • Distinguishing painful Raynaud's mimickers is crucial because they can increase patient stress and lead to unnecessary medical procedures.
  • The review aims to educate on Raynaud's syndrome, identify painful mimickers, and provide updated recommendations for diagnosis and management.
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Article Synopsis
  • Erythromelalgia is a rare and painful condition that affects the hands and feet, causing redness and skin peeling, often triggered by genetic factors, medications, or medical conditions.
  • A case study of a 47-year-old man revealed that his episodes of erythromelalgia were linked to his use of the nasal decongestant pseudoephedrine, which he used intermittently for two years.
  • Stopping the medication led to the complete resolution of his symptoms, highlighting the need for awareness of this potential side effect to improve diagnosis and treatment options.
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Article Synopsis
  • - Erythromelalgia is a painful condition characterized by warmth, redness, and swelling in the skin, often linked to issues with blood vessel control, and has no consistently effective treatments available.
  • - A 36-year-old woman was treated with interosseous membrane stimulation, receiving eight sessions over a year, which significantly alleviated her symptoms for several hours post-treatment.
  • - The results suggest that interosseous membrane stimulation is a promising and safe therapy for erythromelalgia, potentially helping to correct imbalances in the autonomic nervous system affecting the extremities.
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Analgesic effect of Botulinum toxin in neuropathic pain is sodium channel independent.

Neuropharmacology

August 2024

Institute of Neurophysiology, RWTH Aachen University Hospital, Pauwelsstr. 30, 52074, Aachen, Germany; Scientific Center for Neuropathic Pain Research Aachen, SCN(Aachen), RWTH Aachen University Hospital, Pauwelsstr. 30, 52074, Aachen, Germany.

Article Synopsis
  • Botulinum neurotoxin type A (BoNT/A) is being explored as a treatment for neuropathic pain, but its exact mechanism of action is still not well understood.* -
  • Recent research focused on how BoNT/A interacts with voltage-gated sodium channels, particularly Nav1.7 and Nav1.3, using different experimental systems, including human-derived sensory neurons.* -
  • Findings indicated that BoNT/A and its components had limited impact on sodium channel function and did not alter activity in sensory neurons from both healthy individuals and those with a genetic pain condition, suggesting other mechanisms may underlie its pain-relieving effects.*
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Dysautonomia and response to guanfacine in individuals with an SCN9A variant.

Clin Auton Res

April 2024

Department of Neurology, Dysautonomia Center and Autonomic Disorders Division, New York University Langone Health and New York University Grossman School of Medicine, 530 First Av, Suite 9Q, New York, NY, 10016, USA.

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