883 results match your criteria Erythromelalgia


A reappraisal of the presence of small or large fiber neuropathy in patients with erythromelalgia.

Neurophysiol Clin 2021 Apr 19. Epub 2021 Apr 19.

Neurological and Neurovascular Department, Saint Joseph Hospital, Paris, France; Faculty of Medicine, Paris University, France.

Objective: To assess the contribution of large and small nerve fiber alteration in erythromelalgia (EM).

Methods: Thirty-three EM patients were included and underwent clinical evaluation based on EM severity score, DN4, and Utah Early Neuropathy Scale (UENS) score. Neurophysiological evaluation consisted in nerve conduction studies (NCS) for large nerve fibers and specific tests for small nerve fibers: electrochemical skin conductance, cold and warm detection thresholds, and laser evoked potentials. Read More

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Cutaneous reactions reported after Moderna and Pfizer COVID-19 vaccination: A registry-based study of 414 cases.

J Am Acad Dermatol 2021 Apr 7. Epub 2021 Apr 7.

Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts; Medical Practice Evaluation Center, Mongan Institute, Massachusetts General Hospital, Boston, Massachusetts. Electronic address:

Background: Cutaneous reactions after messenger RNA (mRNA)-based COVID-19 vaccines have been reported but are not well characterized.

Objective: To evaluate the morphology and timing of cutaneous reactions after mRNA COVID-19 vaccines.

Methods: A provider-facing registry-based study collected cases of cutaneous manifestations after COVID-19 vaccination. Read More

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Population Pharmacokinetics of Vixotrigine in Healthy Volunteers and Subjects with Trigeminal Neuralgia, Painful Lumbosacral Radiculopathy and Erythromelalgia.

Eur J Drug Metab Pharmacokinet 2021 May;46(3):395-404

Ann Arbor Pharmacometrics Group, Ann Arbor, MI, USA.

Background: Vixotrigine is a voltage and use dependent sodium channel blocker currently under development for treatment of various neuropathic pain indications.

Objective: The objective of this work was to develop a population pharmacokinetic model and assess effects of various covariates on pharmacokinetic parameters of vixotrigine.

Method: Plasma concentration-time data from 12 Phase 1 or 2 studies were included in the analyses. Read More

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Erythromelalgia: A Child With V400M Mutation in the Gene.

Neurol Genet 2021 Apr 1;7(2):e570. Epub 2021 Mar 1.

Stony Brook University Hospital (C.N., L.B.-E.), NY; Pneumophtisiology Clinical Hospital (S.B.), Brasov, Romania; Kings County Hospital (A.M.), Brooklyn, NY; and SUNY Downstate (S.T.), Maimonides Medical Center, Brooklyn.

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Acral vascular syndrome Lennert type T cell lymphoma-a case report.

J Egypt Natl Canc Inst 2021 Mar 4;33(1). Epub 2021 Mar 4.

Department of Geriatric Medicine, All India Institute of Medical Sciences (AIIMS), Room no. 3095A, New Delhi, 110029, India.

Background: Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and erythromelalgia but can be rarely seen in the malignant condition. Patients may present pain, permanent digital blanching or cyanosis, and desquamation or ulceration of the fingers. Acral vascular syndrome is rarely associated with lymphoid neoplasm and is associated with smoking, autoimmune connective tissue diseases, and vasculitis. Read More

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Painful, reappearing eruption in a medically complex 4-year-old.

BMJ Case Rep 2021 Feb 18;14(2). Epub 2021 Feb 18.

Pediatric Hospitalist Medicine, Department of Pediatrics, Duke University, Durham, North Carolina, USA.

A 4-year-old boy with atypical, complete DiGeorge and CHARGE (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities and ear abnormalities) syndromes presented with frequent episodes of a painful, markedly erythematous eruption associated with swelling. Evaluation revealed non-specific findings on skin biopsy at the time of eruption and no pathogenic mutation in the SCN9A gene. The patient was diagnosed with secondary erythromelalgia based on clinical presentation. Read More

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February 2021

Dorsal Root Ganglion Stimulation for Erythromelalgia Related Foot Pain: A Case Report and Review of the Literature.

Pain Pract 2021 Jan 28. Epub 2021 Jan 28.

Division of Pain Medicine, Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Erythromelalgia is a rare neurovascular disorder characterized by erythema, warmth, and episodic burning pain, often felt in the face, hands, and feet. Symptoms are typically worse with heat, exercise, stress, and during the overnight hours. Management often requires a multidisciplinary approach, including pain trigger avoidance, cool water baths, and topical and oral neuropathic medications. Read More

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January 2021

Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms.

Case Rep Vasc Med 2020 9;2020:8887423. Epub 2020 Nov 9.

Division of Experimental Medicine and Immunotherapeutics, University of Cambridge, Cambridge, UK.

Background: Myeloproliferative neoplasms are a heterogeneous group of disorders resulting from the abnormal proliferation of one or more terminal myeloid cells-established complications include thrombosis and haemorrhagic events; however, there is limited evidence to suggest an association with arterial hypertension. Herein, we report two independent cases of severe hypertension in JAK2 mutation-positive myeloproliferative neoplasms. . Read More

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November 2020

Three Cases of Food Poisoning Due to Paralepistopsis acromelalga Diagnosed from an Outbreak of Erythromelalgia.

Intern Med 2020 Dec 22. Epub 2020 Dec 22.

Dokkyo Medical University Hospital, Diagnostic and Generalist Medicine, Japan.

A married couple of a 62-year-old woman and a 64-year-old man as well as their neighbor, an 84-year-old woman, visited the hospital complaining of a burning sensation on their hands and feet that had presented on the same day. They had consumed mushrooms that had been picked on a mountain five days before the onset of the symptoms. The symptoms were attributed to Paralepistopsis acromelalga. Read More

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December 2020

Phosphorylation of a chronic pain mutation in the voltage-gated sodium channel Nav1.7 increases voltage sensitivity.

J Biol Chem 2020 Dec 22. Epub 2020 Dec 22.

Institute of Physiology, RWTH Aachen University Hospital, Germany.

Mutations in voltage-gated sodium channels (Navs) can cause alterations in pain sensation, such as chronic pain diseases like inherited erythromelalgia (IEM). The IEM-causing mutation Nav1.7 p. Read More

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December 2020

Combination gel of 2% amitriptyline and 0.5% ketamine to treat refractory erythromelalgia pain - a case report of pain control success.

Rev Esp Anestesiol Reanim 2021 May 25;68(5):293-296. Epub 2020 Dec 25.

Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; Chronic Pain Unit, Department of Anaesthesiology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Erythromelalgia (EM) is a rare autosomal dominant neuropathy characterized by the combination of severe burning pain and erythematous warm extremities. Chronic pain control is most often unsuccessful and a completely effective therapy is yet to be identified. Recent studies have reported significant improvements in pain management using a combination of amitriptyline and ketamine in a topical formulation. Read More

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Erythromelalgia Caused by Polycythemia Vera Combined with Primary Aldosteronism.

Eur J Case Rep Intern Med 2020 13;7(11):001852. Epub 2020 Aug 13.

Postgraduate Clinical Training Centre, Shimane University Hospital, Shimane, Japan.

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. She had initially visited a dermatologist and was diagnosed with cellulitis and treated with cefditoren pivoxil for 5 days, but there was no improvement. The pain worsened and was accompanied by a burning sensation. Read More

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Successful ultrasound-guided stellate ganglion block treatment in a patient with upper limb erythromelalgia.

Dermatol Ther 2020 11 17;33(6):e14459. Epub 2020 Nov 17.

Department of Anesthesiology, West China Hospital of Sichuan University, Chengdu, China.

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November 2020

[Acrosyndromes (Raynaud's phenomenon, erythermalgia, acrocyanosis, frostbite, digital].

Rev Prat 2020 05;70(5):e147-e153

Service de médecine interne, diabète et maladies métaboliques, clinique médicale B, Hôpitaux universitaires de Strasbourg, 67000 Strasbourg, France.

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Extensive Lumbar Sympathetic Ganglion Block Combined With Epidural Block for Primary Erythromelalgia: A Case Report.

A A Pract 2020 Oct;14(12):e01325

From the Departments of Pain Management and Palliative Care Medicine.

A 19-year-old-woman experienced severe burning pain in the lower extremities with erythema and swelling. She was diagnosed with primary erythromelalgia (PE). The pain was unresponsive to medications but relieved by immersing her feet in cold water. Read More

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October 2020

Autoimmune progesterone dermatitis mimicking facial erythromelalgia successfully treated with hysterectomy and bilateral salpingo-oophorectomy.

JAAD Case Rep 2020 Oct 28;6(10):1104-1106. Epub 2020 Aug 28.

Department of Dermatology, University of Rochester Medical Center, Rochester, New York.

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October 2020

Red Ear Syndrome After Tympanoplasty: A New Association to Add to the List!

Headache 2020 Oct 27;60(9):2081-2082. Epub 2020 Aug 27.

Department of Neurology, Centro Hospitalar Universitário de São João, EPE, Porto, Portugal.

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October 2020

Acute monophasic erythromelalgia pain in five children diagnosed as small-fiber neuropathy.

Eur J Paediatr Neurol 2020 Sep 7;28:198-204. Epub 2020 Jul 7.

Department of Pediatrics, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; University of Lausanne, Lausanne, Switzerland.

The small-fiber polyneuropathies (SFN) are a class of diseases in which the small thin myelinated (Aδ) and/or unmyelinated (C) fibers within peripheral nerves malfunction and can degenerate. SFN usually begins in the farthest, most-vulnerable axons, so distal neuropathic pain and symptoms from microvascular dysregulation are common. It is well known in adults, e. Read More

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September 2020

Uncoupling sodium channel dimers restores the phenotype of a pain-linked Na 1.7 channel mutation.

Br J Pharmacol 2020 10 24;177(19):4481-4496. Epub 2020 Aug 24.

Institute of Physiology, Uniklinik RWTH Aachen University, Pauwelsstrasse 30, Aachen, Deutschland, 52074, Germany.

Background And Purpose: The voltage-gated sodium channel Na 1.7 is essential for adequate perception of painful stimuli. Mutations in the encoding gene, SCN9A, cause various pain syndromes in humans. Read More

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October 2020

Spinal Cord Stimulation in the Treatment of Pediatric Erythromelalgia.

World Neurosurg 2020 10 9;142:388-390. Epub 2020 Jul 9.

Department of Anesthesiology, Pain and Perioperative Medicine, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China. Electronic address:

Background: In children, erythromelalgia is a rare but difficult to manage condition that results in bilateral episodic pain and redness in distal extremities. It is heat intolerant and relieved by cooling. Management of erythromelalgia is difficult and requires a complex multidisciplinary approach. Read More

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October 2020

Acute dysautonomia and erythromelalgia associated with testicular seminoma: A case report.

Rev Neurol (Paris) 2021 Jan-Feb;177(1-2):146-147. Epub 2020 Jul 4.

Service de Neurologie, groupe hospitalier de la région sud Alsace, 20 Avenue du Dr René Laennec, 68100 Mulhouse, France. Electronic address:

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Secondary Erythromelalgia - Case Report.

Actas Dermosifiliogr 2020 Dec 3;111(10):885-886. Epub 2020 Jul 3.

Departamento de Dermatología, Hospital de Santarém EPE, Santarém, Portugal.

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December 2020

Heater-Associated Erythema Ab Igne: Case Report and Review of Thermal-Related Skin Conditions.

Cureus 2020 May 11;12(5):e8057. Epub 2020 May 11.

Dermatology, San Diego Family Dermatology, National City, USA.

Erythema ab igne is a thermal-associated skin condition that can occur secondary to persistent direct or indirect contact with heat. Historically, erythema ab igne has been linked to fireplace and stove exposures; more recently, it has been associated with heaters, hot water bottles, and laptops. A 48-year-old woman presented for the evaluation of hyperpigmented, reticulated macular lesions on her distal legs. Read More

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Red scrotum syndrome: An update on clinicopathologic features, pathogenesis, diagnosis, and management.

J Am Acad Dermatol 2020 Jun 1. Epub 2020 Jun 1.

Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon. Electronic address:

The genital skin may be affected by a variety of dermatoses, be it inflammatory, infectious, malignant, idiopathic, or others. The red scrotum syndrome is characterized by persistent erythema of the scrotum associated with a burning sensation, hyperalgesia, and itching. Its cause is unknown, but proposed mechanisms include rebound vasodilation after prolonged topical corticosteroid use and localized erythromelalgia. Read More

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Primary Erythromelalgia Complicated by Cellulitis: A Case Report and Review of Literature.

Am J Med Case Rep 2020 5;8(6):153-158. Epub 2020 Apr 5.

Department of Internal Medicine, State University of New York, Downstate Health Sciences University, Brooklyn, NY 11203 USA.

Background: Erythromelalgia is a rare disease with increasing incidence. It manifests as episodic painful red extremities triggered by heat. External cooling provides temporary symptomatic relief but may lead to complications such as cellulitis. Read More

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Drug repurposing in Raynaud's phenomenon through adverse event signature matching in the World Health Organization pharmacovigilance database.

Br J Clin Pharmacol 2020 11 14;86(11):2217-2222. Epub 2020 May 14.

Inserm CIC1406, clinical pharmacology department, Grenoble Alpes university hospital, Grenoble, France.

Aims: Several pharmacological treatments are recommended for Raynaud's phenomenon (RP) secondary to systemic sclerosis, but they only have modest efficacy. A way to efficiently identify new drugs is drug repurposing, which can be based on signature matching. The signature could be derived from chemical structures, pharmacological affinity or adverse event profiles. Read More

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November 2020

Heterogeneous presentation of caspr2 antibody-associated peripheral neuropathy - A case series.

Eur J Pain 2020 08 30;24(7):1411-1418. Epub 2020 Apr 30.

Focus Program Translational Neurosciences (FTN), Rhine Main Neuroscience Network (rmn2), Department of Neurology, University Medical Center of the Johannes-Gutenberg University Mainz, Mainz, Germany.

Contactin-associated protein 2-like (caspr2) antibodies have been discovered recently. Since then a multitude of patients with caspr2 antibodies presenting with different neurological symptoms have been reported. Here, we describe three patients with caspr2 antibodies with different types of pain/no pain in combination with peripheral neuropathy. Read More

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