Treatment Of Erythromelalgia Research Articles

Clinical Challenges in Primary Erythromelalgia: a Real-Life Experience from a Single Center and a Diagnostic-Therapeutic Flow-Chart Proposal.

Primary erythromelalgia (EM) is a rare clinical syndrome characterized by recurrent erythema, burning pain and warmth of the extremities. The symptoms greatly compromise the patients' quality of life leading to severe disability. SCN9A mutations can be the cause of the disease. Dermatologists are often the specialists these patients turn to for assistance. To describe the demographic and clinical characteristics of patients with primary EM, to assess the presence and mutation types in the SCN9A gene, to evaluate the effectiveness of several therapeutic approaches, and to propose a diagnostic algorithm with therapeutic implications. A monocentric retrospective study using the database of patients with a discharge diagnosis of primary EM of our Center. Demographic, clinical, instrumental and laboratory data of patients were reviewed. Eleven female patients (age range 16 to 57) were selected. All patients were affected in both the lower and upper extremities. Follow-up ranged from 2 to 9 years. Four patients had four different heterozygous variants of the SCN9A gene. Two patients, although genetically negative, had a suggestive family history. A variety of medications were tried in all our patients to alleviate symptoms, but their efficacy was variable, partial and/or transitory. The most effective therapies were antihistamines, venlafaxine, and mexiletine. The diagnosis and treatment of EM remain challenging. Patients with this condition display a wide spectrum of clinical manifestations and severity, as well as a paucity of resources and structures to support them. Mutations in the SCN9A gene are not always detected.

Surgical treatment of a patient with erythromelalgia (Mitchell’s syndrome) using invasive spinal cord stimulation: A Clinical case

BACKGROUND: Erythromelalgia is a rare hereditary disorder manifesting the basic triad of symptoms: erythro redness, melos limb, and algos pain. It was first described by the American neurologist, S. Weir Mitchell in 1878. Clinical manifestations of the disease worsen the physical and psychological condition of the patient leading to reduced quality of life, increased morbidity and mortality. Currently, etiotropic therapy for erythromelalgia that demonstrates high efficacy in individuals with this pathology, has not been developed. Moreover, there is no consensus on treatment strategies for this category of patients, emphasized by the absence of clinical guidelines for the treatment of erythromelalgia. Treatment of patients with erythromelalgia is currently based on sequential pharmacotherapy in order to select the most effective therapy.
 CLINICAL CASE: We presented the result of surgical treatment of erythromelalgia in a 15-year-old adolescent using invasive spinal cord stimulation.
 DISCUSSION: Erythromelalgia remains an understudied condition with the lack of sufficient understanding of its etiology and pathogenesis. For the first time in Russia, a technique of invasive spinal cord stimulation was used in a pediatric patient with erythromelalgia, which resulted in a significant reduction of neuropathic pain, restoration of vasomotor regulation in the form of reduced edema and hyperemia.
 CONCLUSIONS: In a patient with prolonged and pronounced refractory neuropathic pain caused by erythromelalgia, spinal cord stimulation was the only effective treatment technique alternative to symptomatic and drug therapy. Spinal cord stimulation should be considered as a method of treating neuropathic pain associated with pharmacoresistant forms of erythromelalgia.

502 Treatment of Erythromelalgia and Frostbite: A Case Report

502 Treatment of Erythromelalgia and Frostbite: A Case Report

Topically Applied Midodrine, 0.2%, an α1-Agonist, for the Treatment of Erythromelalgia.

Topically Applied Midodrine, 0.2%, an α1-Agonist, for the Treatment of Erythromelalgia.

Erythromelagia: A Rare and Hard-to-Treat Condition: A 9-Year-Old Boy Responsive to Intravenous Lidocaine and Oral Mexilitene

Dear Editor: We would like to report our successful use of intravenous lidocaine and oral mexiletine in the treatment of erythromelalgia on a 9-year-old boy. One month prior to consult, he experienced progressive, burning pain in hands and feet that was relieved with cold water. The affected extremity demonstrated mild erythema, swelling, and warmth. He arrived to our institution derived from another hospital, reporting a pain intensity of 10/10 on the visual analog scale (VAS) with gabapentin as sole treatment, and no diagnosis to explain these signs and symptoms. Initially, the pediatric unit ordered oral acetaminophen, ketorolac, and pregabaline for neuropathic pain management, but there was no response after 4 days of treatment. Our pain unit evaluated the patient and performed a bolus injection of intravenous lidocaine (1 mg/kg in 20 mL of 0.9% saline over 1 minute), with good response, decreasing his …

Erythema and Burning Pain in the Vulva: A Possible Phenotype of Erythromelalgia

We report a case of burning vulvar pain accompanied by erythema responding to an oral combination of a benzodiazepine and a beta blocker. The positive response to two medication classes used in the treatment of erythromelalgia supports the possibility of a localized manifestation of this disorder in the genital region.

Experience with oral mexiletine in primary erythromelalgia in children

Primary erythromelalgia is characterized by burning pain, redness, and warmth in the extremities. We present two cases of primary erythromelalgia both of whom presented with a history of several months of severe burning pain in both hands and feet. Both patients had received multiple pain medications with no improvement in symptoms. Pain was relieved by putting affected parts in ice cold water, which resulted in immersion injury of the affected parts. Both patients stopped taking part in school and social activities. We tried oral mexiletine, a class Ib antiarrythmic agent, in view of its reported role in various chronic painful conditions. Dramatic improvement was observed with its use. Both patients improved after several weeks of use, and there were fewer soaking episodes. We observed no adverse effects with mexilitine therapy.

Erythromelalgia: two case reports and literature review

Erythromelalgia is a rare disease of unclear etiology characterized by recurrent erythema, burning pain and warmth of the affected extremities. In this paper we reported 2 cases of primary familial erythromelalgia and difficulties with achieving a significant improvement with the currently available treatment. Moreover, this paper provides a brief update on pathophysiology, prognosis and treatment of erythromelalgia.

High-Dose Oral Magnesium Treatment of Chronic, Intractable Erythromelalgia

BACKGROUND:Previous studies with magnesium have shown beneficial effects in pain syndromes and in vascular disorders (hypertension, migraines, Raynaud's phenomenon). However, results have been variable, possibly because of the limited oral doses achievable due to frequent diarrhea.OBJECTIVE: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. Intolerant of several standard magnesium products, he attained high doses of magnesium by taking intravenous-grade magnesium sulfate diluted in water orally (up to 24 mL/d of MgSO4 50% = 1166 mg/d of magnesium).METHODS:After 12 months of continued improvement, patient 1, a board member of The Erythromelalgia Association, notified other members of the success of this therapy via an Internet communication channel. He encouraged those interested in high-dose magnesium therapy to consult their physicians and to report their results to the Association. Twelve patients responded to this request, describing several standard oral magnesium products.RESULTS:Overall, 8 of 13 patients (61.5%) reported improvement (1, remission; 3, major improvement; 2, moderate improvement; 2, mild improvement). Four patients (30.8%) reported no response to magnesium therapy, and 1 patient's symptoms worsened. Two patients' magnesium dose was limited because of diarrhea.CONCLUSIONS:Despite recent progress in understanding and treating EM, this vascular disorder remains painful and life-altering for many patients. In this informal survey, the use of high oral doses of magnesium produced good and sometimes dramatic results in 8 of 13 patients who had been unresponsive to many other treatments. These results suggest a possible role for high-dose oral magnesium in the treatment of EM and, perhaps, other vascular disorders.

Endoscopic Thoracic Sympathectomy for Primary Erythromelalgia in the Upper Extremities

Endoscopic Thoracic Sympathectomy for Primary Erythromelalgia in the Upper Extremities

Erythromelalgia in Essential Thrombocythemia Is Characterized by Platelet Activation and Endothelial Cell Damage but not by Thrombin Generation

Erythromelalgia, a characteristic aspirin-responsive microvascular thrombotic complication in essential thrombocythemia (ET), may develop despite oral anticoagulant treatment or treatment with heparin, suggesting that the generation of thrombin is not a prerequisite for its development. 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. Furthermore, 2 ET patients with a history of erythromelalgia were studied at regular time intervals after discontinuation of aspirin until erythromelalgia recurred. As compared with asymptomatic ET patients and control subjects erythromelalgia was characterized by significantly higher beta-TG and TM levels but no significant differences were detected in either F1 + 2 or TDP levels. Treatment of erythromelalgia with aspirin resulted in disappearance of erythromelalgic signs and symptoms, which was paralleled by a significant decrease of beta-TG and TM levels. Histopathologic and immunohistochemical analysis of biopsies derived from erythromelalgic skin areas of 2 ET patients showed that erythromelalgic thrombi stained positively for von Willebrand factor opposed to only a weak fibrin staining. Our data suggest that erythromelalgia is caused by the intravascular activation and aggregation of platelets with subsequent sludging or occlusion of the acral arterial microvasculature. The generation of thrombin appears not to be essential for the formation of these platelet thrombi, thereby giving a plausible explanation for the inefficacy of coumadin derivatives and heparin in the prevention and treatment of erythromelalgia in essential thrombocythemia.

Platelet Consumption in Thrombocythemia Complicated by Erythromelalgia: Reversal by Aspirin

The involvement of platelets in the pathogenesis of erythromelalgia, a frequent and characteristic microvascular thrombotic manifestation in patients with primary thrombocythemia and polycythemia rubra vera, was investigated by measuring the survival and turnover of 51Cr labeled autologous platelets in 10 patients with thrombocythemia complicated by erythromelalgia, in 10 asymptomatic thrombocythemia patients and in 6 subjects with reactive thrombocytosis. The mean platelet survival time of the erythromelalgia patients was 4.2 +/- 0.2 days, which is significantly decreased as compared with asymptomatic thrombocythemia patients (6.6 +/- 0.3 days, p < 0.001) and patients with reactive thrombocytosis (8.0 +/- 0.4 days, p < 0.001). The mean platelet survival time of asymptomatic thrombocythemia patients was significantly decreased (p < 0.01) as compared with reactive thrombocytosis patients. Treatment of erythromelalgia with aspirin increased the mean platelet survival time from 4.0 +/- 0.3 days to 6.9 +/- 0.4 days (p < 0.001) and was associated with an elevation of the platelet count of 216 +/- 30 x 10(9) platelets per liter (p < 0.001). Coumadin failed to improve platelet survival or symptoms caused by erythromelalgia. The increased platelet consumption in erythromelalgia is attributed to the formation of platelet thrombi in the arterial microvasculature. This conclusion is supported by the ability of aspirin to interrupt platelet consumption and clinical features of erythromelalgia.

The treatment of erythromelalgia depends upon accurate diagnosis

The treatment of erythromelalgia depends upon accurate diagnosis

Arterial Surgery

London's St. Mary's Hospital has for 20 years drawn pilgrims seeking the latest truth on surgery of the arteries. Eastcott's<i>Arterial Surgery</i>has brought together in one place all the lore accumulated by Rob and his disciples. The book remains, however, a personal production of the author himself. It makes a special contribution to the literature of arterial surgery. Authoritative as it travels the highways of the field, it delights the reader by roaming the more interesting byways. Almost nothing is omitted. One can read of the beaded arteries seen on arteriography (although why they are more common in Buerger's disease is not discussed), tuberculous arteritis, the Charcot joints in diabetic neuropathy, the arterial lesions, polymyalgia rheumatica, and the treatment of erythromelalgia by methysergide. One yearns for the leisure one would need to appreciate all the riches in the book. A unique sort of scholarship is evident, a union between