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Erythromelalgia

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https://www.readbyqxmd.com/read/29771216/mindfulness-based-cognitive-hypnotherapy-and-skin-disorders
#1
Philip D Shenefelt
Mindfulness-based cognitive hypnotherapy integrates mindfulness, cognitive-behavioral therapy, and hypnotherapy to improve physical, emotional, mental, and/or spiritual aspects of skin disorders. Meditation, including mindfulness meditation, and hypnosis both utilize trance phenomena to help produce focalization and specific improvements in skin disorders through psycho-neuro-endocrine-immunologic mechanisms. Hypnosis, cognitive hypnotherapy, focused meditation, and mindfulness meditation are discussed with respect to improving various skin disorders including acne, acne excoriée, alopecia areata, atopic dermatitis, congenital ichthyosiform erythroderma, dyshidrotic dermatitis, erythema nodosum, erythromelalgia, furuncles, glossodynia, herpes simplex, hyperhidrosis, ichthyosis vulgaris, lichen planus, neurodermatitis, nummular dermatitis, postherpetic neuralgia, prurigo nodularis, pruritus, psoriasis, rosacea, trichotillomania, urticaria, verruca vulgaris, and vitiligo...
July 2018: American Journal of Clinical Hypnosis
https://www.readbyqxmd.com/read/29534983/cutaneous-manifestations-of-chronic-vascular-disease
#2
REVIEW
Steven M Dean
In the contemporary era of medical diagnosis via sophisticated radiographic imaging and/or comprehensive serological testing, a focused physical examination remains paramount in recognizing the cutaneous manifestations of chronic vascular disease. Recognition of the unique cutaneous signs of lymphatic and venous hypertension assists in the diagnosis as well as the staging and classification of both lymphedema and chronic venous insufficiency. Awareness of explicit dermatologic vasomotor manifestations aids not only in the identification of acrocyanosis, Raynaud phenomenon, pernio, and erythromelalgia but also mitigates confusion related to their clinical overlap...
March 10, 2018: Progress in Cardiovascular Diseases
https://www.readbyqxmd.com/read/29524220/erythromelalgia-improvement-in-pain-with-transcranial-magnetic-stimulation
#3
Leonardo A Tjahjono, Paola Sandroni, Shirlene Sampson, Mark D P Davis
No abstract text is available yet for this article.
March 10, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29402440/-pain-management-in-children-with-erythromelalgia-case-report
#4
Estela Irene Bortoli, Rioko Kimiko Sakata
Erythromelalgia is a neuropathic pain syndrome due to an autosomal dominant gene, characterized by erythema, increased skin temperature and burning pain in hands and feet, whose treatment is often unsatisfactory. In this paper, we report a case of a 9 years old female patient whose first episode of burning pain, erythema and edema of the hands, without triggering factors, had instant relief after immersion in cold water. She presented with systemic arterial hypertension and had seizures. The patient was treated with gabapentin (150mg...
February 3, 2018: Revista Brasileira de Anestesiologia
https://www.readbyqxmd.com/read/29374838/translational-model-systems-for-complex-sodium-channel-pathophysiology-in-pain
#5
Katrin Schrenk-Siemens, Corinna Rösseler, Angelika Lampert
Chronic pain patients are often left with insufficient treatment as the pathophysiology especially of neuropathic pain remains enigmatic. Recently, genetic variations in the genes of the voltage-gated sodium channels (Navs) were linked to inherited neuropathic pain syndromes, opening a research pathway to foster our understanding of the pathophysiology of neuropathic pain. More than 10 years ago, the rare, inherited pain syndrome erythromelalgia was linked to mutations in the subtype Nav1.7, and since then a plethora of mutations and genetic variations in this and other Nav genes were identified...
January 28, 2018: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/29299961/erythromelalgia
#6
REVIEW
Peter Franz Klein-Weigel, Theresa Sophie Volz, Jutta Gisela Richter
Erythromelalgia is a rare syndrome characterized by the intermittent or, less commonly, by the permanent occurrence of extremely painful hyperperfused skin areas mainly located in the distal extremities. Primary erythromelalgia is nowadays considered to be a genetically determined neuropathic disorder affecting SCN9A, SCN10A, and SCN11A coding for NaV1.7, NaV1.8, and NaV1.9 neuronal sodium channels. Secondary forms might be associated with myeloproliferative disorders, connective tissue disease, cancer, infections, and poisoning...
February 2018: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/29206554/front-line-treatment-options-for-chronic-phase-chronic-myeloid-leukemia
#7
Neil P Shah
The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice...
January 20, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29152720/secondary-erythromelalgia-a-tryptophan-dietary-supplement-induced-case-associated-with-elevated-5-hydroxyindoleacetic-acid-5hiaa-urinary-levels
#8
Andrea Michelerio, Federica Derlino, Valeria Brazzelli, Camilla Vassallo
No abstract text is available yet for this article.
January 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29121832/how-a-simple-ankle-sprain-turned-into-neuropathic-pain-complex-reflex-sympathetic-dystrophy-versus-erythromelalgia
#9
Ann Regina Lurati
A 36-year-old woman sustained a Grade 2 ankle sprain at work. Two days after the injury, the ankle and foot became red and she complained of "intense burning pain." First diagnosed with complex reflex sympathetic dystrophy, the employee was prescribed medications that provided some pain relief; a subsequent temporary nerve block provided additional relief. However, the symptoms returned and she was treated unsuccessfully with surgical sympathectomy. The employee was referred to a neurologist and diagnosed with primary erythromelalgia, a rare pain disorder that can be mistaken as complex reflex sympathetic dystrophy...
November 1, 2017: Workplace Health & Safety
https://www.readbyqxmd.com/read/28990532/a-novel-scn9a-mutation-f826y-in-primary-erythromelalgia-alters-the-excitability-of-nav1-7
#10
B Wu, Y Zhang, H Tang, M Yang, H Long, G Shi, J Tang, X Shi
BACKGROUND: Primary erythromelalgia (PE) is a dominant inherited disorder characterized by recurrent pain, redness, and warmth of the extremities that is caused by gain-of-function mutations in Nav1.7 encoding gene SCN9A. Most of the PE-causing mutations of Nav1.7 have been shown to be able to render Nav1.7-expressing cells hyperexcitable, however in most PE cases the symptoms are refractory to treatment with sodium channel blockers and the mechanism underlying the intractability has not been clearly clarified...
2017: Current Molecular Medicine
https://www.readbyqxmd.com/read/28791051/a-unique-case-for-spinal-cord-stimulation-successful-treatment-of-small-fiber-neuropathy-pain-using-multiple-spinal-cord-stimulators
#11
Maxim Eckmann, Alexander Papanastassiou, Mark Awad
Spinal cord stimulators have commonly been used to treat multiple pain conditions. This case report represents a unique case of using multiple spinal cord stimulators for widespread small fiber neuropathy pain. This case report concerns patient JJ who first presented with generalized neuropathic pain. His pain was an intermittent burning, stinging quality that originally focused in both of his feet and progressed to include his legs and arms and eventually involved his entire body. The pain would last moments to hours at least daily...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28751508/between-fire-and-ice-refractory-hypothermia-and-warmth-induced-pain-in-inherited-erythromelalgia
#12
See Wan Tham, Li Li, Philip Effraim, Stephen Waxman
Inherited erythromelalgia (IEM) is a well-described pain disorder caused by mutations of sodium channel Nav1.7, a peripheral channel expressed within dorsal root ganglion and the sympathetic ganglion neurons. Clinically, IEM is characterised by paroxysmal attacks of severe pain, usually in the distal extremities, triggered by warmth or exercise. Pain is not adequately treated by existing pharmacological agents. Individuals with IEM classically cool their limbs for relief, in some cases resulting in tissue injury...
July 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28658526/reverse-pharmacogenomics-carbamazepine-normalizes-activation-and-attenuates-thermal-hyperexcitability-of-sensory-neurons-due-to-nav-1-7-mutation-i234t
#13
Yang Yang, Talia Adi, Philip R Effraim, Lubin Chen, Sulayman D Dib-Hajj, Stephen G Waxman
BACKGROUND AND PURPOSE: Pharmacotherapy for pain currently involves trial and error. A previous study on inherited erythromelalgia (a genetic model of neuropathic pain due to mutations in the sodium channel, Nav 1.7) used genomics, structural modelling and biophysical and pharmacological analyses to guide pharmacotherapy and showed that carbamazepine normalizes voltage dependence of activation of the Nav 1.7-S241T mutant channel, reducing pain in patients carrying this mutation. However, whether this approach is applicable to other Nav channel mutants is still unknown...
June 28, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28541877/erythromelalgia-involving-the-face
#14
Rachel R Gilmore, Danielle S Applebaum, Jessica L Parsons, Sylvia Hsu
Erythromelalgia is a rare disorder characterized by burning pain, erythema, and increased temperature typically involving the distal extremities. Although it can progress to involve the face, erythromelalgia presenting only on the face is particularly rare. This disorder is often misdiagnosed when it presents on the extremities and is even more likely to be misdiagnosed when presenting only on the face, delaying appropriate treatment and causing considerable frustration for the patient. We report a case of a 26-year-old woman with erythromelalgia that involved only the face for a number of years and was treated unsuccessfully as rosacea, seborrheic dermatitis, and contact dermatitis...
April 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28490900/complex-management-of-a-patient-with-refractory-primary-erythromelalgia-lacking-a-scn9a-mutation
#15
Sarah A Low, Wendye Robbins, Vivianne L Tawfik
A 41-year-old woman presented with burning and erythema in her extremities triggered by warmth and activity, which was relieved by applying ice. Extensive workup was consistent with adult-onset primary erythromelalgia (EM). Several pharmacological treatments were tried including local anesthetics, capsaicin, ziconotide, and dantrolene, all providing 24-48 hours of relief followed by symptom flare. Interventional therapies, including peripheral and sympathetic ganglion blocks, also failed. Thus far, clonidine and ketamine have been the only effective agents for our patient...
2017: Journal of Pain Research
https://www.readbyqxmd.com/read/28418213/pain-thresholds-supra-threshold-pain-and-lidocaine-sensitivity-in-patients-with-erythromelalgia-including-the-i848tmutation-in-nav-1-7
#16
T Helås, D Sagafos, I P Kleggetveit, H Quiding, B Jönsson, M Segerdahl, Z Zhang, H Salter, M Schmelz, E Jørum
OBJECTIVES: Nociceptive thresholds and supra-threshold pain ratings as well as their reduction upon local injection with lidocaine were compared between healthy subjects and patients with erythromelalgia (EM). METHODS: Lidocaine (0.25, 0.50, 1.0 or 10 mg/mL) or placebo (saline) was injected intradermally in non-painful areas of the lower arm, in a randomized, double-blind manner, to test the effect on dynamic and static mechanical sensitivity, mechanical pain sensitivity, thermal thresholds and supra-threshold heat pain sensitivity...
April 18, 2017: European Journal of Pain: EJP
https://www.readbyqxmd.com/read/28413058/erythromelalgia-identification-of-a-corticosteroid-responsive-subset
#17
Gabriel L Pagani-Estévez, Paola Sandroni, Mark D Davis, James C Watson
BACKGROUND: Corticosteroids (CS) may benefit certain patients with erythromelalgia. OBJECTIVES: Our objective was to determine clinical predictors of corticosteroid-responsive erythromelalgia. METHODS: Patients with erythromelalgia who received CS were identified and stratified into corticosteroid nonresponders (NRs), partial corticosteroid responders (PSRs), complete corticosteroid responders (CSRs), and steroid responders (SRs = PSRs + CSRs)...
March 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28410768/-small-fiber-neuropathy
#18
V Langlois, A-L Bedat Millet, M Lebesnerais, S Miranda, F Marguet, Y Benhamou, P Marcorelles, H Lévesque
Small fiber neuropathy (SFN) is still unknown. Characterised by neuropathic pain, it typically begins by burning feet, but could take many other expression. SFN affects the thinly myelinated Aδ and unmyelinated C-fibers, by an inherited or acquired mechanism, which could lead to paresthesia, thermoalgic disorder or autonomic dysfunction. Recent studies suggest the preponderant role of ion channels such as Nav1.7. Furthermore, erythromelalgia or burning mouth syndrome are now recognized as real SFN. Various aetiologies of SFN are described...
February 2018: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28381558/gain-of-function-mutation-of-a-voltage-gated-sodium-channel-na-v-1-7-associated-with-peripheral-pain-and-impaired-limb-development
#19
Brian S Tanaka, Phuong T Nguyen, Eray Yihui Zhou, Yong Yang, Vladimir Yarov-Yarovoy, Sulayman D Dib-Hajj, Stephen G Waxman
Dominant mutations in voltage-gated sodium channel NaV 1.7 cause inherited erythromelalgia, a debilitating pain disorder characterized by severe burning pain and redness of the distal extremities. NaV 1.7 is preferentially expressed within peripheral sensory and sympathetic neurons. Here, we describe a novel NaV 1.7 mutation in an 11-year-old male with underdevelopment of the limbs, recurrent attacks of burning pain with erythema, and swelling in his feet and hands. Frequency and duration of the episodes gradually increased with age, and relief by cooling became less effective...
June 2, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28235406/network-topology-of-nav1-7-mutations-in-sodium-channel-related-painful-disorders
#20
Dimos Kapetis, Jenny Sassone, Yang Yang, Barbara Galbardi, Markos N Xenakis, Ronald L Westra, Radek Szklarczyk, Patrick Lindsey, Catharina G Faber, Monique Gerrits, Ingemar S J Merkies, Sulayman D Dib-Hajj, Massimo Mantegazza, Stephen G Waxman, Giuseppe Lauria
BACKGROUND: Gain-of-function mutations in SCN9A gene that encodes the voltage-gated sodium channel NaV1.7 have been associated with a wide spectrum of painful syndromes in humans including inherited erythromelalgia, paroxysmal extreme pain disorder and small fibre neuropathy. These mutations change the biophysical properties of NaV1.7 channels leading to hyperexcitability of dorsal root ganglion nociceptors and pain symptoms. There is a need for better understanding of how gain-of-function mutations alter the atomic structure of Nav1...
February 24, 2017: BMC Systems Biology
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