Erythromelalgia (Erythermalgia)

A peripheral arterial disease that is characterized by the triad of ERYTHEMA, burning PAIN, and increased SKIN TEMPERATURE of the extremities (or red, painful extremities). Erythromelalgia may be classified as primary or idiopathic, familial or non-familial. Secondary erythromelalgia is associated with other diseases, the most common being MYELOPROLIFERATIVE DISORDERS.

Also Known As:

Networked: 209 relevant articles (20 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pain (Aches)
2.	Erythromelalgia (Erythermalgia)
3.	Thrombocytosis (Thrombocythemia)
4.	Essential Thrombocythemia
5.	Transient Ischemic Attack

Experts

1.	Waxman, Stephen G: 26 articles (01/2015 - 09/2004)
2.	Dib-Hajj, Sulayman D: 19 articles (05/2013 - 09/2004)
3.	Drenth, Joost P H: 9 articles (12/2012 - 06/2005)
4.	Tyrrell, Lynda: 9 articles (01/2012 - 03/2006)
5.	Lampert, Angelika: 7 articles (03/2015 - 11/2006)
6.	Davis, Mark D P: 6 articles (09/2015 - 01/2002)
7.	Sandroni, Paola: 5 articles (09/2015 - 01/2002)
8.	Han, Chongyang: 5 articles (04/2014 - 03/2006)
9.	Cummins, Theodore R: 5 articles (10/2011 - 09/2004)
10.	Yang, Yong: 5 articles (12/2009 - 03/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Erythromelalgia:

1.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 01/01/1989 - "Alternatively, a single daily dose of aspirin results in dramatic improvement in most patients with either primary or secondary erythromelalgia." 09/01/2012 - "The purpose of this report was to describe the case of a child presenting with premature loss of primary teeth and the difficulties in determining the final diagnosis of erythromelalgia, which responded positively to low-grade aspirin therapy." 01/01/2012 - "Erythromelalgia: an uncommon presentation precipitated by aspirin withdrawal." 04/01/2006 - "Peculiar microcirculatory disturbances such as erythromelalgia and visual and hearing symptoms also commonly occur in these patients, and are highly responsive to aspirin. " 11/01/2005 - "Her erythromelalgia immediately disappeared following interventional therapy along with aspirin. "
2.	Amitriptyline (Elavil)FDA LinkGeneric 03/01/1993 - "Erythromelalgia: association with hereditary sensory neuropathy and response to amitriptyline." 03/01/2013 - "The objective of this study was to assess the response of erythromelalgia to compounded topical amitriptyline-ketamine. " 03/01/2013 - "We retrospectively evaluated 36 patients with erythromelalgia who were treated with compounded topical amitriptyline-ketamine from January 1, 2004, through January 31, 2011. " 03/01/2013 - "Topical amitriptyline combined with ketamine for the treatment of erythromelalgia: a retrospective study of 36 patients at Mayo Clinic." 03/01/2006 - "Combination gel of 1% amitriptyline and 0.5% ketamine to treat refractory erythromelalgia pain: a new treatment option?"
3.	KetamineFDA LinkGeneric 11/01/2002 - "[Successful intravenous administration of low dose ketamine for pain caused by erythromelalgia: report of a case]." 03/01/2013 - "The objective of this study was to assess the response of erythromelalgia to compounded topical amitriptyline-ketamine. " 03/01/2013 - "We retrospectively evaluated 36 patients with erythromelalgia who were treated with compounded topical amitriptyline-ketamine from January 1, 2004, through January 31, 2011. " 03/01/2013 - "Topical amitriptyline combined with ketamine for the treatment of erythromelalgia: a retrospective study of 36 patients at Mayo Clinic." 03/01/2006 - "Combination gel of 1% amitriptyline and 0.5% ketamine to treat refractory erythromelalgia pain: a new treatment option?"
4.	Nifedipine (Adalat)FDA LinkGeneric 04/01/1989 - "The lack of overall efficacy of nifedipine was attributable to a high incidence of side effects, including an unusual symptom complex resembling erythromelalgia. " 05/01/1996 - "Because the patient was not taking any other medications and the symptoms started with the administration of nifedipine and were relieved after its discontinuation, nifedipine was thought to be the cause of the erythromelalgia-like syndrome." 05/01/1996 - "Several nifedipine-related reports describe an erythromelalgia-like syndrome similar to our reported case. " 05/01/1996 - "To describe a patient who was diagnosed with Raynaud's phenomenon, was prescribed immediate-release nifedipine, and developed a possible erythromelalgia-like syndrome. " 05/01/1996 - "Possible erythromelalgia-like syndrome associated with nifedipine in a patient with Raynaud's phenomenon."
5.	Mexiletine (Mexitil)FDA LinkGeneric 09/01/2004 - "A case of primary erythromelalgia improved by mexiletine." 10/01/2014 - "The aim of this study was, therefore, to investigate the biophysical effects of mexiletine on the L858F primary erythromelalgia NaV 1.7 mutation in vitro. " 03/01/2011 - "We present an unusual presentation of erythromelalgia of the ears in a patient who has been refractory to multiple therapies and in whom relief of symptoms was achieved with the use of mexiletine. " 03/01/2011 - "Refractory erythromelalgia of the ears: response to mexiletine." 07/01/2009 - "Experience with oral mexiletine in primary erythromelalgia in children."
6.	Type A Botulinum Toxins (Botulinum A Toxin)FDA Link 05/01/2013 - "Botulinum toxin type A might be safe and effective as an alternative treatment for refractory erythromelalgia, but further large-scale studies are required."
7.	Nitroprusside (Sodium Nitroprusside)FDA LinkGeneric 01/01/1984 - "The symptoms of two children with erythromelalgia were relieved promptly by the intravenous administration of sodium nitroprusside (1-5 micrograms/kg/min). " 09/01/2002 - "Erythromelalgia: an endothelial disorder responsive to sodium nitroprusside." 05/01/1997 - "The pathogenesis of erythromelalgia is unclear and precludes formulating a proposed mechanism by which nitroprusside has benefit in children. " 05/01/1997 - "This case adds to existing literature substantiating the benefit of nitroprusside for the treatment of erythromelalgia in pediatric patients. " 05/01/1997 - "No relapse of erythromelalgia was seen when nitroprusside was discontinued and the patient remained well after 6 months. "
8.	CalciumIBA 01/01/2005 - "On account of these properties, calcium antagonists have also been used for the prevention and treatment of various dermatologic diseases such as erythromelalgia, idiopathic- or CREST-related calcinosis cutis, primary and secondary Raynaud phenomenon, chilblains, chronic anal fissures, keloids, and burn scars. " 02/01/2002 - "To describe the case of a 53-year-old white man (the author) with disabling erythromelalgia (EM) who achieved modest improvement limited by adverse effects with calcium antagonists and then obtained remission with the use of magnesium. " 01/01/2004 - "The most useful oral medications for erythromelalgia seem to be aspirin, propranolol, clonazepam, cyproheptadine, drugs inhibiting serotonin re-uptake (venlafaxine and sertraline), tricyclic antidepressants (amitriptyline, imipramine), anticonvulsants (gabapentin), calcium antagonists (nifedipine, diltiazem), and prostaglandins (micoprostol). "
9.	Prostaglandin-Endoperoxide Synthases (Cyclooxygenase)IBA 04/01/2003 - "Thus, cyclooxygenase metabolites are necessary for erythromelalgia to develop. " 03/01/2006 - "The broad spectrum of aspirin-sensitive erythromelalgia, its microvascular ischemic complications, migraine-like atypical or typical transient ischemic attacks (cerebral and ocular) as well as acute coronary syndromes in thrombocythemia vera (essential thrombocythemia and thrombocythemia associated with polycythemia vera in maintained remission by phlebotomy) is caused by platelet cyclo-oxygenase-mediated arteriolar inflammation, fibromuscular intimal proliferation without and with occlusive thrombosis by platelet-rich thrombi in the end-arterial microvasculature of the peripheral, cerebral, ocular and coronary circulation. "
10.	MethylprednisoloneFDA LinkGeneric 01/01/2009 - "High-dose then slowly tapered methylprednisolone resulted in rapid remission of painful erythromelalgia and complete electrophysiological recovery. "

Therapies and Procedures

1.	Aftercare (After-Treatment) 10/01/1991 - "The symptoms of adults with erythromelalgia are markedly relieved after treatment with a single daily dose of acetylsalicylic acid. " 07/01/1995 - "Erythromelalgia is causally related to thrombocythaemia; prompt relief of painful symptoms after treatment with aspirin is typical." 09/01/1996 - "To study this, a cross-sectional comparison of the plasma levels of thrombomodulin (TM), platelet factor 4 (PF4), beta-thromboglobulin (beta-TG), prothrombin fragment 1 + 2 (F1 + 2) and total degradation products of fibrin(ogen) (TDP) was made between 5 ET patients suffering from erythromelalgia, 16 asymptomatic ET patients and 20 control subjects, and after treatment with aspirin, respectively. "
2.	Sympathectomy (Sympathectomies) 11/01/2010 - "Long-term remission of primary erythermalgia with R1150W polymorphism in SCN9A after chemical lumbar sympathectomy." 11/01/2004 - "Successful thoracoscopic sympathectomy for primary erythromelalgia in the upper extremities." 04/01/1999 - "Endoscopic thoracic sympathectomy for primary erythromelalgia in the upper extremities."
3.	Massage (Reflexology) 01/01/2010 - "The present report evaluates massage as a complementary therapy to reduce pain and other symptoms associated with erythromelalgia. " 01/01/2010 - "Massage therapy techniques as pain management for erythromelalgia: a case report." 01/01/2010 - "In this patient with erythromelalgia, effleurage and petrissage as massage therapy techniques provided temporary pain relief in the lower extremities and long-term benefits that relieved anxiety, which improved restorative sleep and increased the patient's participation in activities of daily living. "
4.	Hypnosis (Mesmerism) 03/01/2000 - "A wide spectrum of dermatologic disorders may be improved or cured using hypnosis as an alternative or complementary therapy, including acne excoriée, alopecia areata, atopic dermatitis, congenital ichthyosiform erythroderma, dyshidrotic dermatitis, erythromelalgia, furuncles, glossodynia, herpes simplex, hyperhidrosis, ichthyosis vulgaris, lichen planus, neurodermatitis, nummular dermatitis, postherpetic neuralgia, pruritus, psoriasis, rosacea, trichotillomania, urticaria, verruca vulgaris, and vitiligo. "
5.	Activities of Daily Living (ADL) 01/01/2010 - "A 31-year-old female with a long-standing history of erythromelalgia bilaterally in the lower extremities presented with complaints of acute pain exacerbation, anxiety, decreased quality of sleep, and difficulty with activities of daily living for prolonged periods of time. " 01/01/2010 - "In this patient with erythromelalgia, effleurage and petrissage as massage therapy techniques provided temporary pain relief in the lower extremities and long-term benefits that relieved anxiety, which improved restorative sleep and increased the patient's participation in activities of daily living. "