Presence of specific IgG antibody to the A type inclusions of erythromelalgia-related poxvirus in the sera of patients with epidemic erythromelalgia.
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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↗
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↗
Erythromelalgia in thrombocythemia of various myeloproliferative disorders.
Erythromelalgia is caused by platelet-mediated acral inflammation and arteriolar thrombosis in thrombocythemia in its primary form or associated with polycythemia vera. The prompt and lasting relief of burning pain by low-dose aspirin is a prerequisite for the diagnosis of thrombocythemic erythromelalgia. Here we extend observations on the occurrence of erythromelalgia in thrombocythemia associated with primary myelofibrosis, Philadelphia-chromosome positive micromegakaryocytic myelofibrosis, and myelodysplastic syndrome type II. It is concluded that erythromelalgia may occur in thrombocythemia of all variants of chronic myeloproliferative disease as well as myelodysplastic syndrome if platelet counts are sufficiently high.
Adult↗
Erythromelalgia in a patient with thrombotic thrombocytopenic purpura.
Erythromelalgia is characterized by paroxysmal burning pain and redness of the distal extremities; primary and secondary forms exist. Secondary erythromelalgia is most commonly associated with essential thrombocythemia and polycythemia vera, and the pathogenic mechanism is thought to be platelet aggregation. Thrombotic thrombocytopenic purpura is characterized by thrombocytopenia, microangiopathic anemia, renal failure, and fluctuating neurologic signs and symptoms. The pathogenic mechanism suggested for thrombotic thrombocytopenic purpura is diffuse platelet aggregation. We describe a female patient in whom thrombotic thrombocytopenic purpura was associated with erythromelalgia. This has not been previously reported.
Aged↗
Thrombocythaemic erythromelalgia in chronic myeloproliferative and myelodysplastic disorders.
Erythromelalgia, which is specific for primary thrombocythaemia or polycythaemia with thrombocythaemia, is reported in a case of primary myelofibrosis at platelet counts of between 350 and 450 X 10(9)/l. In addition, the unexpected occurrence of thrombocythaemic erythromelalgia associated with Ph1 chromosome positive micromegakaryocytic myelofibrosis and with myelodysplastic syndrome type II is described. Therefore it is concluded that erythromelalgia may occur in all variants of myeloproliferative disease as well as myelodysplastic syndrome as long as they present with thrombocythaemia.
Adult↗
Pathogenesis of erythromelalgia.
A defective prostaglandin metabolism in patients with erythromelalgia may explain several of the clinical features of this condition, such as the red discoloration and burning sensations of the skin. In two patients with erythromelalgia a grossly abnormal bullous reaction to intradermally injected PGE1, PGE2, and PGF1alpha occurred, whereas a normal reaction appeared after injection of histamine, serotonin, and bradykinin. Furthermore, prostaglandin-like material was detected in increased concentration in skin perfusates from these patient. In PGE1-equivalents the concentration amounted to 2.0 and 3.2 ng/ml of the original perfusate, as compared to 0.1 ng/ml in normal skin. The capacity of synthesizing prostaglandins was increased in skin biopsy material from both patients. At least part of the therapeutic effect of aspirin in these patients may be due to the influence of this drug on prostaglandin metabolism.
Adult↗
Erythromelalgia--the role of hypnotherapy.
Erythromelalgia is an unusual condition characterized by attacks of burning pain in the hands and feet with local congestion and increased skin temperature. We report a case of erythromelalgia, with transient hypertension and elevated urinary catecholamines successfully treated by hypnotherapy. Such an association has not to our knowledge been previously reported in English language publications.
Adolescent↗
Stereotactic surgery of erythromelalgia.
Erythromelalgia is a very rare vascular disease affecting children and is accompanied by severe burning pain in the extremities. The disease is practically incurable. There are no recommendations in the literature concerning the possibility of managing the disease by stereotactic surgery. We operated on 3 children with severe pain due to erythromelalgia. The stereotactic destruction of VPL and CM was performed (in 2 cases on one side and bilaterally in 1). An excellent result with the disappearance of not only pain but all signs of the disease was noted in all cases (follow-up 8, 4.5 years and 6 months).
Adolescent↗
Primary erythromelalgia: the role of skin sympathetic nerve activity.
A 54-year-old man complained of burning pain, warm skin and erythema in his extremities. A diagnosis of primary erythromelalgia was made. Microneurography was used to clarify the role of skin sympathetic nerve activity in the pathophysiology of primary erythromelalgia. The patient showed normal skin sympathetic nerve activity but no vasoconstriction response. Aspirin activated the skin sympathetic nerve activity and improved vasoconstriction producing symptomatic relief. These results suggest that the lack of vasoconstriction following vasoconstrictor activity of the skin sympathetic nerves results in increased skin blood flow and burning pain.
Aspirin↗
[Primary erythromelalgia].
Erythromelalgia is a rare disease characterized by intense erythema, burning pain and increased temperature in the distal of the extremities. Primary forms and secondary forms have been described, most commonly with essential thrombocythemia and policythemia vera. The authors describe a fifteen year old patient with primary erythromelalgia and discuss the pathogenic, clinical and therapeutic features of this disease.
Adolescent↗
[Erythromelalgia in adults. Apropos of 16 cases].
The authors report sixteen consecutive cases of erythromelalgia, an infrequent disease in which local heat, redness, and pain develop in the hands and/or feet in recurrent attacks. The disease was essential in nine patients; in the remaining seven, the cause was a myeloproliferative syndrome (polycythemia vera in 3 cases and thrombocythemia in 1 case) or a drug (bromocriptine, nicardipine, and nifedipine, one case each). Acetylsalicylic acid was effective in only six of the nine essential cases. Intravascular platelet activation and aggregation with plugging of the arterioles has been suggested as the mechanism of erythromelalgia in patients with myeloproliferative disorders. Other, as yet unelucidated pathophysiologic events underlie the juvenile-onset forms, which usually fail to respond to acetylsalicylic acid.
Adult↗
[A case of primary erythromelalgia (erythermalgia) treated with neural blockade].
A case of primary erythromelalgia which was treated successfully with lumbar sympathetic block and total spinal block (TSB) is reported. The patient was a 21-year-old woman with 18-year history of pain, burning, swelling, redness and warm sensation in both feet and lower part of the legs that caused the patient to soak her feet and legs frequently in ice cold water in order to obtain pain relief. The patient had been treated with a variety of medications including aspirin, indomethacin, methysergide maleate, and carbamazepine with no relief. Recently, the excessive exposure to cold water had caused extensive immersion foot (trench foot) with secondary infection (fusarium infection). Treatment with bilateral lumbar sympathetic block had markedly improved the symptom. Furthermore, treatment with TSB against causalgic state was performed 8 times for 4 months. During this period, the patient experienced the symptom which was much milder than those before treatment with TSB. Lumbar sympathetic block and TSB are useful methods for treatment of primary erythromelalgia.
Adult↗
[A case-control study on epidemic erythromelalgia].
A case-control study on a erythromelalgia was conducted in a stratified random sample of 1633 middle school students in Zao Yang county, Hubei province. Among them 462 and 1,191 students were in a case group and a control group, respectively. Data were analysed by Mental-Haenszal method and multiple regression analysis. It was showed that erythromelalgia was significantly associated with residents students, cold living condition, malnutrition and disfunction of vegetative nerve system. The proper preventive measures should be tamen to the teenager, especially to the female and resident students of the same age group.
Adolescent↗
Erythromelalgia.
Erythromelalgia is an extraordinary disease which remains elusive in its pathophysiology and management. Victims suffer intense burning and redness of the hands and feet. In what appears to be the antithesis of Raynaud's disease, the pain is relieved by emersion in cold. A child with erythromelalgia is described whose symptoms began at age 3 years. Pharmacological management trials and thermography are incorporated in the report.
Child, Preschool↗
Autosomal dominant erythromelalgia.
We present a kindred of 29 persons affected with erythromelalgia (erythermalgia) in 5 generations. This paper updates the family reported by Burbank et al. [1966]. Patients have symptoms of intermittent intense burning limb pain related to increased skin temperature. No successful treatment has been identified, and the pathogenetic mechanism has not been established. Most affected individuals are female.
Adult↗
Further characterization of the biological and pathogenic properties of erythromelalgia-related poxviruses.
Six isolates of erythromelalgia-related poxvirus (ERPV) were characterized with respect to host range, c.p.e. and inclusions, pock formation on chorioallantoic membrane (CAM), morphogenesis, serological reactivity, pathogenesis in animals and DNA restriction fragment profile. The results suggest that ERPV is either a new member of the Orthopoxvirus genus or a subspecies of ectromelia virus. Evidence is provided that (i) ERPV has a wide host range in vitro in which characteristic viral c.p.e. and inclusion bodies are induced; (ii) ERPV, unlike ectromelia virus, causes the formation of tiny greyish-white pocks on CAM both at 34 degrees C and 39 degrees C; (iii) eosinophilic A-type inclusions of ERPV do not contain viral particles; (iv) ERPV isolates are neutralized by both rabbit anti-vaccinia virus and mouse anti-ectromelia virus sera, but not vice versa; (v) young rabbits are not susceptible to ERPV by skin and/or corneal scratch infection even though ERPV is lethal for mice by intraperitoneal inoculation; (vi) the HindIII and SalI fragment profiles of ERPV P-4 DNA are similar to, but obviously different from, those of Chinese ectromelia virus. These biological and pathogenic characteristics of ERPV are distinguishable from those of other members of the genus Orthopoxvirus currently described in the literature.
Animals↗
Erythromelalgia.
Erythromelalgia, characterized by temperature-dependent redness, pain, and warmth in one or more extremities, may be a primary disease or occur secondarily to underlying illnesses. Myeloproliferative disorders (eg, essential thrombocythemia) and a number of other associations have been reported. Two cases are described: one associated with essential thrombocythemia and the other the first reported case associated with pernicious anemia.
Aged↗