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At least 19 recordsLinked to original sources
PubMed2003

Erythromelalgia: vasculopathy, neuropathy, or both? A prospective study of vascular and neurophysiologic studies in erythromelalgia.

OBJECTIVE: To assess the frequency and type of vascular changes and neurologic abnormalities in patients with erythromelalgia. DESIGN: Prospective study of patients with no spontaneous symptoms at the time of their visit and with provoked symptoms. SETTING: Tertiary referral center. PATIENTS: Sixty-seven patients presenting with erythromelalgia at Mayo Clinic, Rochester, Minn, from 1999 through 2001. INTERVENTIONS: Testing nerve and vascular function in patients without symptoms present; testing vascular function after provoking symptoms with exercise or by increasing ambient temperature. MAIN OUTCOME MEASURES: In patients in whom symptoms could be elicited, vascular function with and without symptoms was assessed by measurement of local skin temperature, laser Doppler flow, and transcutaneous oximetry. Neurologic assessment included electromyography, nerve conduction studies, and autonomic reflex screening (using the quantitative sudomotor axon reflex test, adrenergic function testing, heart rate response to deep breathing, and the Valsalva ratio). RESULTS: Autonomic reflex screening was performed on 57 (85%) of the 67 patients. Of these 57 patients, 46 (81%) had abnormal quantitative sudomotor axon reflex test results; 14 (25%) had abnormal adrenergic function; and 15 (26%) had abnormal cardiovagal function. Put in another way, results were abnormal for 49 (86%) of the 57 patients who had autonomic reflex screening. Severe sudomotor abnormalities (ie, absent or markedly reduced sweat production) were present in 46 (94%) of these 49 patients; 14 (29%) had abnormal adrenergic function, and 15 (31%) had a cardiovagal abnormality. Electromyography and nerve conduction studies were performed in 24 (36%) of the 67 patients. Of these 24 patients, 14 (58%) had abnormal electromyographic results and 10 (42%) had abnormal nerve conduction study results. Vascular function studies, with and without symptoms present, were performed in 13 of the 67 patients. During symptoms, the mean temperature of the toe skin increased by 7.8 degrees C, and blood flow increased 10.2-fold. Paradoxically, mean transcutaneous oximetry measurements did not change. CONCLUSION: This prospective study extends and confirms our previous observation that, in addition to other forms of neuropathy, most patients with erythromelalgia have small-fiber neuropathy.

Adolescent↗
PubMed1989

Erythromelalgia and myeloproliferative disorders.

Erythromelalgia (erythermalgia) is characterized by attacks of severe burning pain, erythema, and warmth of the extremities, primarily the feet and, to a lesser extent, the hands. The distress is provoked by environmental heat, exercise, and dependency; it is relieved by exposure to cold and elevation of the extremity. Primary and secondary forms of erythromelalgia exist. Secondary erythromelalgia has been linked to a wide variety of diseases, the most common of which are certain myeloproliferative disorders: polycythemia vera and essential thrombocythemia. We describe, for the first time, a patient in whom chronic myelogenous leukemia was associated with the development of erythromelalgia, review the 60 cases in the world literature of erythromelalgia in patients with myeloproliferative syndromes, and compare the primary and secondary forms of the disease. Importantly, symptoms of erythromelalgia preceded the onset of a myeloproliferative disease by a median of 2 1/2 years. Therefore, all patients with erythromelalgia should be monitored with periodic blood cell counts. An abnormal hemoglobin level, white blood cell or platelet count, or immature cells in the differential count are not seen in idiopathic erythromelalgia and should alert the physician to the possibility of a more serious underlying disease process. Treatment of the myeloproliferative syndrome will sometimes alleviate the symptoms of erythromelalgia. Alternatively, a single daily dose of aspirin results in dramatic improvement in most patients with either primary or secondary erythromelalgia.

Erythromelalgia↗
PubMed1997

Nitroprusside treatment of erythromelalgia in an adolescent female.

OBJECTIVE: To report a case of erythromelalgia in an adolescent patient successfully treated with nitroprusside. CASE SUMMARY: A 15-year-old girl with erythromelalgia resistant to aspirin therapy received an infusion of nitroprusside. The response of the erythromelalgia to nitroprusside was dramatic, with complete pain resolution within 17 hours after the start of therapy. No relapse of erythromelalgia was seen when nitroprusside was discontinued and the patient remained well after 6 months. DISCUSSION: This case adds to existing literature substantiating the benefit of nitroprusside for the treatment of erythromelalgia in pediatric patients. Erythromelalgia in children may represent a different disease entity than that seen in adults, which is commonly responsive to aspirin therapy. The pathogenesis of erythromelalgia is unclear and precludes formulating a proposed mechanism by which nitroprusside has benefit in children. CONCLUSIONS: Nitroprusside is valuable for erythromelalgia resistant to aspirin therapy in pediatric patients. Because of unanswered questions regarding the disease, aspirin remains the agent of first choice in all patients with this rare disease.

Adolescent↗
PubMed1996

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.

Adult↗
PubMed1991

Erythromelalgia: review of clinical characteristics and pathophysiology.

Erythromelalgia is characterized by burning discomfort, warmth, and dermal erythema of the feet and/or the hands. Lowering the involved part and exposure to heat worsen symptoms, whereas elevation or cooling of the extremity relieves the discomfort. Several different subtypes of erythromelalgia have been documented and include an adult-onset form secondary to myeloproliferative syndrome-related thrombocytosis and an early-onset form that appears in childhood or adolescence and is idiopathic. A disturbed platelet function affecting the microvasculature has been implicated in thrombocythemia-related erythromelalgia. Importantly, manifestations of erythromelalgia often precede the onset of the myeloproliferative disease by several years. Therefore, the blood cell count should be monitored periodically in all adult patients with erythromelalgia. An abnormal hemoglobin, white blood cell, or platelet count or the presence of immature cells in the differential should prompt the physician to initiate a diagnostic evaluation for an underlying hematologic disorder. The symptoms of adults with erythromelalgia are markedly relieved after treatment with a single daily dose of acetylsalicylic acid. In contrast, childhood erythromelalgia appears without an underlying disorder, may be familial, and is resistant to treatment with aspirin.

Adolescent↗
PubMed2001

Hot feet: erythromelalgia and related disorders.

Erythromelalgia is an extraordinary pain syndrome first described by S. Weir Mitchell in 1878. Episodes of severe burning pain in the distal limbs, accompanied by striking redness and warmth of the skin, are precipitated by heat or activity and can be terminated only by cooling the affected part. Primary erythromelalgia is a sporadic or autosomal-dominant hereditary disorder whose symptoms begin in childhood. Secondary erythromelalgia occurs in association with thrombocythemia, collagen-vascular diseases, diabetes mellitus, peripheral neuropathy, and use of certain drugs. Aspirin is effective for patients with thrombocythemia, but most other cases are very resistant to treatment. The pathogenesis of erythromelalgia has remained puzzling, especially the peculiar switch-like manner in which symptoms are turned on by heat and turned off by cold. Following Ochoa's description of the ABC (angry backfiring C nociceptors) syndrome, it seems plausible to regard erythromelalgia as a problem of sensitized skin polymodal C-fiber receptors. C-fiber threshold to activation by heat would be lowered to 32 degrees C to 36 degrees C; activated C fibers would cause vasodilation via axon reflexes with redness, heat, and swelling. Cooling would bring the nociceptors below threshold. Secondary erythromelalgia may result from humoral factors released from platelets or ischemic tissues or from C-fiber injury in some cases of neuropathy, whereas primary erythromelalgia could be due to a mutation of the capsaicin receptor.

Erythromelalgia↗
PubMed1995

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.

Adult↗
PubMed2000

Microvascular arteriovenous shunting is a probable pathogenetic mechanism in erythromelalgia.

Erythromelalgia is a condition consisting of red, warm, and burning painful extremities. Symptoms are relieved by cold and aggravated by heat. A wide variety of etiologic conditions can cause erythromelalgia, but one common pathogenetic mechanism, microvascular arteriovenous shunting, has been hypothesized. The aim of this study was to test this hypothesis. Quantification of skin microvascular perfusion using laser Doppler perfusion imaging and skin temperature at rest and after central body heating was performed in 14 patients with erythromelalgia and 11 controls. Attacks of erythromelalgia were induced in eight patients after heat provocation. In the plantar region of the foot, the location of numerous anatomical arteriovenous shunts, these patients significantly increased the skin perfusion as compared with asymptomatic patients with erythromelalgia and controls. In the dorsal region with few arteriovenous shunts no significant differences between the groups were demonstrated. The results show a relation between clinical symptoms and increased perfusion in the region of numerous anatomical arteriovenous shunts, and support the hypothesis of increased thermoregulatory arteriovenous shunt flow during attacks in primary erythromelalgia.

Adult↗
PubMed2000

Erythromelalgia: new theories and new therapies.

Erythromelalgia is a rare condition that has remained an enigma diagnostically and therapeutically for decades. It has been assumed that erythromelalgia, which is characterized by hot, red, intensely painful feet or hands, may be the opposite of Raynaud's phenomenon. However, new research suggests that these two disorders are more similar than dissimilar. Erythromelalgia usually follows a chronic, sometimes progressive and disabling course. New evidence suggests that this may not be a disease entity at all, but a syndrome of dysfunctional vascular dynamics; recent studies demonstrate that this dysfunction is reversible in some patients. This review article presents the latest theories and successful treatments for erythromelalgia, and data from a survey of members of The Erythromelalgia Association, which was formed to provide information about erythromelalgia to doctors and patients.

Capsaicin↗
PubMedDate not supplied

Erythromelalgia: diagnosis and classification.

Erythromelalgia is not a commonly recognized or diagnosed condition that affects the lower extremities. The first reported case was in 1878, when Mitchell suggested the term "erythromelalgia." This condition is characterized by a burning sensation with erythema of the involved extremity. When the extremity is lowered, or heat is applied, the pain is intensified. The application of cold or elevation of the extremity will have the opposite effect of decreasing the pain. Erythromelalgia is classified as primary or idiopathic if there is no accompanying disease process. Secondary erythromelalgia is associated commonly with myeloproliferative syndrome-related thrombocythemia, and is mostly evident in adult onset of the condition. Treatment for adults with erythromelalgia includes a single daily dose of aspirin, but children who have no associated underlying disorder find little to no relief with acetylsalicylic acid.

Adult↗
PubMed2002

Impaired neurogenic control of skin perfusion in erythromelalgia.

Erythromelalgia is a clinical diagnosis characterized by erythema, increased temperature and burning pain in acral skin. The pain is relieved by cooling and aggravated by warming. The symptoms have been hypothesized to be caused by skin hypoxia due to increased arteriovenous shunting. We examined skin microvascular perfusion in response to vasoconstrictory and vasodilatory stimuli, to characterize local and central neurogenic reflexes as well as vascular smooth muscle and vascular endothelial function, using laser Doppler perfusion measurements in 14 patients with primary erythromelalgia and healthy control persons. Skin perfusion preceding provocative stimuli was significantly reduced in patients with erythromelalgia (p < 0.01). The laser Doppler flowmetry signal after sympathetic stimulation of reflexes mediated through the central nervous system, was significantly diminished in patients with erythromelalgia as compared with healthy controls (Valsalva's maneuver p < 0.01; contralateral cooling test p < 0.05). Local neurogenic vasoconstrictor (venous cuff occlusion and dependency of the extremity) and vasodilator reflexes (local heating of the skin), as well as tests for vascular smooth muscle and vascular endothelial function (postocclusive hyperemic response) were maintained. These results indicate that postganglionic sympathetic dysfunction and denervation hypersensitivity may play a pathogenetic role in primary erythromelalgia, whereas local neurogenic as well as endothelial function is unaffected.

Adolescent↗
PubMed1996

Possible erythromelalgia-like syndrome associated with nifedipine in a patient with Raynaud's phenomenon.

OBJECTIVE: To describe a patient who was diagnosed with Raynaud's phenomenon, was prescribed immediate-release nifedipine, and developed a possible erythromelalgia-like syndrome. CASE SUMMARY: A 24-year-old white woman with a history of esophageal spasms and Raynaud's phenomenon was prescribed nifedipine 10 mg po qid. Approximately 1 hour after the patient had taken the fourth dose of nifedipine, she experienced acute erythema and a burning sensation in her feet and lower limbs, light-headedness, and palpitations. Because of a reportedly abnormally low blood pressure, the patient took diphenhydramine 50 mg po and proceeded to the clinic. On arrival, abnormal vital signs were BP 140/48 mm Hg and HR 130 beats/min. Without any other medical intervention, approximately 30 minutes later her blood pressure and heart rate had returned to baseline at 122/60 mm Hg and 96 beats/min, respectively. The nifedipine was permanently discontinued and the patient's symptoms completely resolved over 24 hours. DISCUSSION: The characteristic symptoms of erythromelalgia include burning pain, increased skin temperature, and erythema of the extremities, usually to the feet, lower legs, and, less often, the hands. Erythromelalgia-like syndromes secondary to the administration of many medications have been reported. Several nifedipine-related reports describe an erythromelalgia-like syndrome similar to our reported case. CONCLUSIONS: 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.

Adult↗
PubMed1985

Erythromelalgia caused by platelet-mediated arteriolar inflammation and thrombosis in thrombocythemia.

Erythromelalgia was the presenting symptom in 26 of 40 patients with thrombocythemia in its primary form or when associated with polycythemia vera. The localized painful burning, redness, and warm congestion in the extremities could be accurately documented with thermography. Skin punch biopsy samples taken from the affected areas showed typical arteriolar inflammation, fibromuscular intima proliferation, and thrombotic occlusions. Erythromelalgia often progressed to ischemic acrocyanosis or necrosis in toes or fingers. Complete relief of pain and restoration of microvascular circulation disturbances was obtained with the cyclo-oxygenase inhibitors aspirin and indomethacin, but not with sodium-salicylate or the platelet inhibitors dipyridamole, sulfinpyrazone, ticlopidine, and dazoxiben. The erythromelalgia was alleviated during busulfan-induced remissions of thrombocythemia and its recurrence coincided with relapsing thrombocythemia. These observations suggest a causal relationship between erythromelalgia and thrombocythemia, in which platelet-mediated inflammatory and occlusive arteriolar changes play a part in the etiology of erythromelalgia.

Adult↗
PubMed2004

Erythromelalgia.

Erythromelalgia is a rare poorly understood clinical condition characterized by intense burning pain, pronounced erythema, and increased skin temperature. Although there are many classifications of the disease, it can basically be divided into primary, which begins spontaneously at any age, and secondary, which is associated with myeloproliferative disorders-related thrombocythemia, polycythemia, collagen-vascular diseases, diabetes mellitus, peripheral neuropathy, autoimmune and infectious diseases, and use of certain medicaments. A wide variety of etiological conditions can cause erythromelalgia, all having a single common pathogenetic mechanism - microvascular arteriovenous shunting. The disease is characterized by severe pain associated with redness and hotness in extremities. The diagnosis is based on the medical history and clinical findings. 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). Erythromelalgia is usually chronic, sometimes progressive, and disabling disease, which can greatly affect the quality of life. Some patients have stable disease and get better, or even experience full resolution of the disease, with time. This review article presents the etiological basis, diagnostics, and therapy of erythromelalgia.

Diagnosis, Differential↗
PubMed1994

[Erythromelalgia: a familial case. Discussion on the role of mercury].

Erythromelalgia is an acrosyndrome characterized by paroxysmal manifestations associating erythema, local sensations of warmth and pain which improve with exposure to cold. Childhood forms, exceptional and usually primary, are quite severe and particularly resistant to treatment. The search for a causal agent is most often negative and the pathogenesis remains to be determined. We report a case of erythromelalgia observed in a 4-year-old girl, her father and her younger sister. This case was particular due to an association with mercury poisoning. The symptomatology was improved after different therapeutic attempts including Clomipramide and, particularly effective, rerigerating socks (D(r) Comet, CNES, Toulouse). In the literature we were unable to find any case of erythromelalgia related to mercury poisoning. The cases of familial erythromelalgia reported suggest X-linked dominant transmission. Finally, this case demonstrated the difficulties in diagnosing and treating erythromelalgia, especially in the child.

Acrodermatitis↗
PubMed2002

Erythromelalgia.

No treatment is consistently effective in the management of patients with erythromelalgia. There is a dearth of adequate studies examining the response of erythromelalgia to therapy. Most recommendations are suggested based on case reports, small case series, and anecdotal reports. The management of erythromelalgia is difficult and frequently involves a multidisciplinary approach. An approach to management of individuals with erythromelalgia includes patient education, learning to avoid episodes relieving discomfort of the episodes, controlling secondary and underlying factors, and use of drugs used to control erythromelalgia.

Journal Article↗
PubMed2000

Natural history of erythromelalgia: presentation and outcome in 168 patients.

OBJECTIVE: To describe the demographics, presentation, and outcome in patients with erythromelalgia--a rare and poorly understood clinical syndrome defined by the triad of red, hot, painful extremities. DESIGN: Retrospective medical record review with follow-up by survey questionnaire. SETTING: Large tertiary care medical center. SUBJECTS: Patients with erythromelalgia examined at the Mayo Clinic, Rochester, Minn, between 1970 and 1994. INTERVENTION: The medical records of 168 patients were analyzed. Follow-up data, which consisted of answers to 2 survey questionnaires or the most recent information in the medical record from patients still alive and death certificates or reports of death for those deceased patients, were obtained for all but 13 patients. MAIN OUTCOME MEASURES: Survival, morbidity, and quality of life. RESULTS: All patients were white; 122 (72.6%) were female, and 46 (27.4%) were male. At presentation, the patients' mean age was 55.8 years (age range, 5-91 years). Symptoms had been present since childhood in 7 patients (4.2%). Six patients (3.6%) had a first-degree relative with erythromelalgia. Symptoms were intermittent in 163 patients (97.0%) and constant in 5 (3.0%). Symptoms predominantly involved feet (148 patients [88.1%]) and hands (43 patients [25.6%]). Kaplan-Meier survival curves revealed a significant decrease in survival compared with that expected in persons of similar age and of the same sex (P<.001). After a mean follow-up of 8.7 years (range, 1.3-20 years), 30 patients (31.9%) reported worsening of, 25 (26.6%) no change in, 29 (30.9%) improvement in, and 10 (10.6%) complete resolution of the symptoms. On a standard health status questionnaire, scores for all but one of the health domains were significantly diminished in comparison with those in the US general population. CONCLUSION: Erythromelalgia is a syndrome with significantly increased mortality and morbidity compared with the US general population.

Adolescent↗