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Results for “ERYTHROMELALGIA”

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At least 73 records · Page 4Linked to original sources

PubMed1984

Histopathology of erythromelalgia in thrombocythaemia.

Erythromelalgia always appears to be an expression of thrombocythaemia which may be provoked by intravascular platelet aggregation, because it is relieved by agents interfering with platelet prostaglandin synthesis. Biopsies were taken from affected areas 1-3 weeks after discontinuation of aspirin. At that time arteriolar changes were present: the endothelial cells were often swollen with large nuclei. Narrowing of the lumen occurred by proliferation of smooth muscle cells with vacuolisation and swelling of the cytoplasm and deposition of intercellular material. The internal elastic lamina appeared to be split between the proliferated cells. This gave rise to the appearances of fibromuscular intimal arteriolar proliferation which were often occluded by thrombi of differing age. Ultimately the arterioles become completely fibrosed. These vascular changes are restricted to arterioles, are partly reversible with aspirin treatment and seem to be a characteristic of erythromelalgia.

Adult↗

PubMed2003

The use of regional anaesthetic blockade in a child with recurrent erythromelalgia.

Primary erythromelalgia is a rare condition, which is characterised by redness, burning pain, and increased temperature of the extremities. We describe a 6 year old boy with symptoms of erythromelalgia and the difficulty surrounding treatment of this condition. Severe pain responded to the use of regional anaesthetic blocks.

Analgesia, Epidural↗

PubMed1992

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↗

PubMed1990

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↗

PubMed2002

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.

Administration, Oral↗

PubMedDate not supplied

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↗

PubMedDate not supplied

Erythromelalgia--a thrombotic complication in chronic myeloproliferative disorders.

Erythromelalgia is a very specific, thrombotic syndrome related with thrombocythemia that may occur during the course of chronic myeloproliferative disorders (MPD), especially polycythemia vera (PV) and essential thrombocythemia (ET). This poorly understood clinical syndrome is characterized by red, congested distal extremities and painful burning sensations, usually confined to the ball of the foot and one or more toes or fingers. If left untreated, it may progress towards acrocyanosis and even peripheral gangrene. Sometimes, it may precede the diagnosis of MPD by months or years. The pathophysiological aspects of erythromelalgia as well as its differentiation with erythermalgia have been reviewed in this study.

Chronic Disease↗

PubMedDate not supplied

[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↗

PubMed1992

[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↗

PubMed2005

Erythromelalgia misdiagnosed as cellulitis.

This case report examines the presentation of a patient with erythromelalgia that was misdiagnosed as cellulitis on several prior occasions. The presentation of bilateral acral edema and erythema, especially in the setting of myeloproliferative and/or connective tissue diseases, should alert the physician to the possibility of alternate diagnoses, including erythromelalgia. Treatments target symptom alleviation, as well as diagnosis and treatment of causative factors.

Cellulitis↗

PubMed1989

[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↗

PubMed1989

[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↗

PubMedDate not supplied

[Surgical treatment of erythromelalgia].

Two children (an 11-year-old boy and a 15-year-old girl) with erythromelalgia and uncontrollable pain in the lower extremities were treated by stereotaxic destruction of VPL and CM and (in one case) by partial rhizotomy. Pain in the extremities and the clinical manifestations of erythromelalgia disappeared after surgical treatment (follow-up periods of 5 and 2 years, respectively). The mechanism of the therapeutic effect of these operations is still not clear. Similar cases are not described in the literature.

Adolescent↗

PubMed1988

Erythromelalgia associated with acute diabetic neuropathy: an unusual condition.

Erythromelalgia is a syndrome characterized by erythema, burning pain and increased skin temperature of the affected extremities. It can be either primary (idiopathic) or secondary, but both forms require a critical temperature between 32-36 degrees C to provoke the crisis. Among the secondary forms, its association with diabetes mellitus is infrequent and its significance is little known. The histological studies have been fundamentally performed in patients with this syndrome affected by thrombocythemia; vascular thrombosis and fibromuscular intimal arteriolar proliferation were the prominent findings not corroborated in patients with the primary form. We studied one patient with acute diabetic neuropathy who developed erythromelalgia. We noted the absence of histopathological lesions, suggesting a disorder in vascular motility regulation favored by the severe neuropathy, without being able to discount a possible immunomediated mechanism. We describe the favourable evolution of the patient after treatment with aspirin and methysergide.

Acute Disease↗

PubMed1977

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↗

PubMedDate not supplied

Erythromelalgia associated with hypertension and leukocytoclastic vasculitis in a child.

Erythromelalgia is an acrocyanotic rheumatic disease presenting with erythema, and pain and a burning sensation in the hands and feet; it is rarely encountered during childhood. Hot or warm conditions may precipitate pain and erythema in the extremities and the symptoms may regress upon the application of cold water. The disease is usually secondary to other systemic diseases in adults. On the other hand, it is idiopathic in children. This article describes a case of erythromelalgia presenting with leukocytoclastic vasculitis and hypertension in a 7-year-old child who responded to therapy with prednisolone and phenoxybenzamine.

Antihypertensive Agents↗

PubMed1992

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↗

PubMed1995

[Cutaneous manifestations of essential thrombocythemia. Erythromelalgia, ischemic acrocyanosis, livedo racemosa].

A case report is presented to illustrate the cutaneous manifestations in essential thrombocythaemia. Knowledge of the related skin lesions may lead to earlier detection of this myeloproliferative disorder. Treatment may prevent severe haemorrhagic or thrombotic complications. Erythromelalgia is causally related to thrombocythaemia; prompt relief of painful symptoms after treatment with aspirin is typical.

Aged↗