911 results match your criteria Erythromelalgia


If your ears are burning we must be talking about red ear syndrome: A brief report.

Australas J Dermatol 2022 May 4. Epub 2022 May 4.

Dermatology Department, Perth Children's Hospital, Nedlands, Western Australia, Australia.

Red Ear Syndrome is an uncommon disorder that can affect all age groups. It is frequently referred to Dermatology as it can present similarly to erythromelalgia. Although the exact pathophysiology is unknown, a common hypothesis suggests a shared pathophysiological background with migraine due to their well-known association. Read More

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Part II Cutaneous Manifestations of Peripheral Vascular Disease.

J Am Acad Dermatol 2022 Apr 30. Epub 2022 Apr 30.

Department of Dermatology, Boston University School of Medicine, Boston, MA; Department of Dermatology, US Department of Veteran Affairs, Boston Health Care System, Boston, MA. Electronic address:

In this Part 2 of a 2-part continuing medical education series, we review the epidemiology of peripheral vascular disease, its association with cutaneous symptoms, and the diagnosis and evaluation of cutaneous features of vascular disorders. As peripheral vascular disease becomes more prevalent globally, it is essential for dermatologists to become competent at accurately recognizing and diagnosing cutaneous manifestations and directing individuals to receive appropriate care and treatment. Read More

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A stepwise approach for the management of primary erythromelalgia: A prospective single-arm study.

J Am Acad Dermatol 2022 Apr 21. Epub 2022 Apr 21.

Department of Dermatology, Peking University First Hospital, Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, National Clinical Research Center for Skin and Immune Diseases, Beijing, China; Dermatology Hospital, Southern Medical University, Guangzhou, China; Department of Dermatology, Xiamen Chang Gung Hospital, Xiamen, China. Electronic address:

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Intravenous Ketamine Infusion as an Adjunctive Pain Treatment for Erythromelalgia: A Pediatric Case Report.

A A Pract 2022 Apr 13;16(4):e01582. Epub 2022 Apr 13.

From the Division of Dermatology, Phoenix Children's Hospital, Phoenix, Arizona.

Erythromelalgia is a rare neurovascular pain condition characterized by erythematous, warm, and painful extremities. Symptoms are exacerbated by heat and relieved by cooling. Treatment is challenging and focuses on symptom control with various medications and therapies targeted toward eliminating destructive cooling behaviors. Read More

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Premature Loss of Deciduous Teeth as a Symptom of Systemic Disease: A Narrative Literature Review.

Int J Environ Res Public Health 2022 03 13;19(6). Epub 2022 Mar 13.

Department of Paediatric Dentistry, Medical University of Warsaw, Binieckiego 6 Street, 02-097 Warsaw, Poland.

Background: Premature loss of primary teeth can occur as a consequence of dental trauma, neonatal tooth extraction, early childhood caries, or periodontal problems, or it can be a manifestation of systemic disease. This review aims to present systemic disorders that can lead to premature loss of deciduous teeth in children and to provide a comprehensive resource for clinical practice for both physicians and dentists.

Methods: This study is a narrative review of original studies and case reports published in English and Polish between 1957 and 2021 that was conducted by searching electronic scientific resources: PubMed, Google Scholar, Web of Science, and Science Direct. Read More

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Inhibition of sodium conductance by cannabigerol contributes to a reduction of dorsal root ganglion neuron excitability.

Br J Pharmacol 2022 Mar 16. Epub 2022 Mar 16.

Department of Neurology, Yale University School of Medicine, New Haven, Connecticut, USA.

Background And Purpose: Cannabigerol (CBG), a non-psychotropic phytocannabinoid and a precursor of ∆ -tetrahydrocannabinol and cannabidiol, has been suggested to act as an analgesic. A previous study reported that CBG (10 μM) blocks voltage-gated sodium (Na ) currents in CNS neurons, although the underlying mechanism is not well understood. Genetic and functional studies have validated Na 1. Read More

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Ragged cuticles and erythematous hands and feet in a pediatric patient with chronic diarrhea.

JAAD Case Rep 2022 Apr 19;22:18-20. Epub 2022 Jan 19.

Department of Dermatology and Dermatologic Surgery, Charleston, South Carolina.

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The Case of the Red Extremities.

Clin Pract Cases Emerg Med 2022 Feb;6(1):83-84

Mayo Clinic, Department of Emergency Medicine, Phoenix, Arizona.

Case Presentation: A 37-year-old man with severe obstructive sleep apnea presented to the emergency department with burning pain, redness and swelling in his hands and feet, worsening for several weeks. Pertinent laboratory studies revealed polycythemia.

Discussion: Erythromelalgia is a clinical diagnosis characterized by painful burning, erythema, warmth, and edema usually involving the distal extremities. Read More

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February 2022

Post-operative erythromelalgia treated with calcium channel blockers.

J Dtsch Dermatol Ges 2022 Mar 19;20(3):347-349. Epub 2022 Feb 19.

Department of Orthopedics, Kasr Alainy Medical School, Cairo University, Cairo, Egypt.

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A Case of Pediatric Auricular Erythromelalgia.

Cureus 2022 Jan 10;14(1):e21088. Epub 2022 Jan 10.

Otolaryngology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, USA.

Erythromelalgia is a rare clinical syndrome involving erythema, warmth, and burning pain in one or more of the extremities. Primary erythromelalgia is idiopathic and can begin during childhood or adulthood. In the pediatric population, auricular erythromelalgia is rare, and its etiology is not well understood. Read More

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January 2022

Stem cell-derived sensory neurons modelling inherited erythromelalgia: normalization of excitability.

Brain 2022 Jan 28. Epub 2022 Jan 28.

Department of Neurology, Yale School of Medicine, New Haven, CT, USA.

Effective treatment of pain remains an unmet healthcare need that requires new and effective therapeutic approaches. NaV1.7 has been genetically and functionally validated as a mediator of pain. Read More

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January 2022

Genetic aspects of pain and its variability in the human population.

Ann Agric Environ Med 2021 Dec 31;28(4):569-574. Epub 2021 Mar 31.

Faculty of Social Medicine, Medical University, Łódź, Poland.

The sensation of pain is common to both animals and human beings. Its threshold, intensity, tolerability, and characteristics are variable and depend on ethnicity, gender, stress exposure, co-existing mental disorders, such as depression or anxiety, social and economical background, as well as on genetic factors. It is estimated that about 5 and 20 percent of population suffer from acute and chronic pain, respectively, which results in the search for medical advice in healthcare facilities, and causes great expenses in health care budgets worldwide. Read More

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December 2021

Successful treatment of knee erythromelalgia with topical oxymetazoline.

Clin Exp Dermatol 2022 Apr 4;47(4):778-780. Epub 2022 Jan 4.

Department of Dermatology, Hospital Universitari Sagrat Cor, Grupo Hospitalario Quironsalud, Barcelona, Spain.

Erythromelalgia is an infrequent syndrome with a profound impact on quality of life. Its management is usually challenging and multiple treatments have been reported with variable response rates. To the best of our knowledge, we present the first case of erythromelalgia successfully treated with topical oxymetazoline. Read More

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Life-threatening hypothermia in a child with primary erythromelalgia.

Pediatr Dermatol 2022 Jan 9;39(1):135-136. Epub 2021 Dec 9.

Division of Pediatric Dermatology, Nationwide Children's Hospital, Columbus, Ohio, USA.

Primary erythromelalgia is a rare autosomal-dominant condition due to pathogenic variant in the SCN9A gene, characterized by childhood onset of excruciating pain, redness, and warmth of acral sites. Patients often resort to ice water baths and other cooling measures to manage the discomfort. Hypothermia is a rare complication, reported only twice previously. Read More

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January 2022

Eltrombopag-associated erythromelalgia in idiopathic thrombocytopenic purpura.

Br J Dermatol 2022 03 24;186(3):585-586. Epub 2021 Nov 24.

Pharmacovigilance Department, Grenoble Alpes University Hospital, Grenoble, F-38000, France.

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Cutaneous findings following COVID-19 vaccination: review of world literature and own experience.

J Eur Acad Dermatol Venereol 2022 Feb 2;36(2):172-180. Epub 2021 Nov 2.

European Center for Angioscience (ECAS), Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.

There is growing evidence that not only the novel coronavirus disease (COVID-19) but also the COVID-19 vaccines can cause a variety of skin reactions. In this review article, we provide a brief overview on cutaneous findings that have been observed since the emerging mass COVID-19 vaccination campaigns all over the world. Unspecific injection-site reactions very early occurring after the vaccination are most frequent. Read More

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February 2022

Fish Oil and BCQ™ as a Novel Treatment Approach to Primary Erythromelalgia: A Case Study.

Integr Med (Encinitas) 2021 Aug;20(4):40-45

National University of Natural Medicine (NUNM) Health Centers, Portland, Oregon.

Erythromelalgia (EM) is a rare disorder, of which the pathophysiology is poorly understood. Though primary EM is thought to be an autosomal-dominant inherited disorder or of idiopathic origin, secondary EM is associated, most often, with myeloproliferative diseases. Pain management is the primary therapeutic target, however, individual patient responses vary widely, and no single treatment has been determined to be effective. Read More

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Cutaneous manifestations of small fibre polyneuropathy.

J Eur Acad Dermatol Venereol 2022 Jan 15;36(1):100-107. Epub 2021 Oct 15.

Harvard Medical School, Boston, MA, USA.

Background: Because typical and atypical features of small fibre polyneuropathy (SFN) in the skin have not been fully elucidated, the diagnosis is often made by the exclusion of alternative conditions rather than by its identification as a primary syndrome.

Objective: The objective of this study was to characterize dermatologic manifestations in patients with SFN.

Methods: Large retrospective series of biopsy-proven SFN cases seen at the Massachusetts General Hospital and Brigham and Women's Hospital (January 2000 to December 2019). Read More

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January 2022

variants and pain modulation: a missense variant in Kv7.3 contributes to pain resilience.

Brain Commun 2021 8;3(3):fcab212. Epub 2021 Sep 8.

Department of Neurology, Yale University School of Medicine, New Haven, CT 06520, USA.

There is a pressing need for understanding of factors that confer resilience to pain. Gain-of-function mutations in sodium channel Nav1.7 produce hyperexcitability of dorsal root ganglion neurons underlying inherited erythromelalgia, a human genetic model of neuropathic pain. Read More

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September 2021

Erythromelalgia associated with dermatomyositis: A case series.

JAAD Case Rep 2021 Oct 18;16:37-40. Epub 2021 Aug 18.

Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

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October 2021

Clinical and pathologic correlation of cutaneous COVID-19 vaccine reactions including V-REPP: A registry-based study.

J Am Acad Dermatol 2022 01 10;86(1):113-121. Epub 2021 Sep 10.

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 COVID-19 vaccination have been commonly reported; however, histopathologic features and clinical correlations have not been well characterized.

Methods: We evaluated for a history of skin biopsy all reports of reactions associated with COVID-19 vaccination identified in an international registry. When histopathology reports were available, we categorized them by reaction patterns. Read More

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January 2022

High frequency dorsal column spinal cord stimulation for management of erythromelalgia.

BMJ Case Rep 2021 Aug 5;14(8). Epub 2021 Aug 5.

Anesthesiology, University of Kansas Medical Center, Kansas City, Kansas, USA.

Erythromelalgia is a rare hereditary channelopathy affecting the Nav1.7 sodium channel. Patients afflicted with this condition suffer from pain in their hands and feet, with vasomotor changes including flushing and redness to the distal upper and lower extremities. Read More

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Erythromelalgia: Pain and Redness of the Feet after Warm Water Exposure.

Intern Med 2022 Jan 17;61(2):279. Epub 2021 Jul 17.

Immuno-Rheumatology Center, St. Luke's International Hospital, Japan.

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January 2022

Erythermalgia (erythromelalgia). Redefining the classification of adult erythromelalgia.

J Med Vasc 2021 Jul 2;46(4):159-162. Epub 2021 Jun 2.

Vascular Medicine Department, Saint-Joseph Hospital, Paris, France.

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Successful use of phlebotomy to treat severe secondary polycythemia due to chronic lung disease.

Hematol Rep 2021 Jun 9;13(2):8961. Epub 2021 Jun 9.

Department of Medicine, West Virginia University, Morgantown, WV, USA.

Secondary polycythemia (SP) occurs as a result of increase erythropoietin levels most commonly as a result of tissue hypoxia. Symptoms such as erythromelalgia, pruritis, and bleeding, which are frequently seen in polycythemia vera (PV), do not commonly occur in SP. Phlebotomy is considered one of the mainstays of therapy for PV but is rarely used for treatment of SP due to concern about worsening tissue hypoxia. Read More

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CT-guided Chemical Lumbar Sympathectomy in the Treatment of Cold Hypersensitivity in the Hands and Feet.

Pain Physician 2021 07;24(4):E459-E466

First Affiliated Hospital of Jiaxing University, Jiaxing, Zhejiang Province, China.

Background: Cold hypersensitivity in the hands and feet is a common clinical symptom in Asian women. Currently, treatment of cold hypersensitivity in the hands and feet is still limited to traditional Chinese medicine, mainly herbal medicine. However, many patients with cold hypersensitivity in the hands and feet in China are not satisfied with the therapeutic effect of herbal medicine, and took medication for a longer time. Read More

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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 (Engl Ed) 2021 05 5;68(5):293-296. Epub 2021 May 5.

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

Neurophysiol Clin 2021 Aug 20;51(4):349-355. Epub 2021 Apr 20.

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 07 7;85(1):46-55. 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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