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PubMed2004

Successful thoracoscopic sympathectomy for primary erythromelalgia in the upper extremities.

Erythromelalgia is known as a rare syndrome of unknown etiology, characterized by redness with burning pain, edema associated with increased skin temperature in the upper and/or lower extremities. Various treatments such as drug therapies and sympathetic blockade were reported. We report two cases including a 57-year-old woman and a 64-year-old woman, showing the successful clinical outcome by bilateral thoracoscopic sympathectomy.

Erythromelalgia↗
PubMed1993

Control of intractable pain in erythromelalgia by using spinal cord stimulation.

A 69-yr-old woman with severe, long-standing erythromelalgia possibly secondary to multiple deep-vein thromboses, was treated with transcutaneous electrical nerve stimulation for the burning pain in her legs and feet. Problems developed, and she was subsequently successfully managed with spinal cord stimulation. The relief was reproduced after a 6-mo period of no stimulation by reestablishing spinal cord stimulation.

Aged↗
PubMedDate not supplied

A refractory case of erythromelalgia involving the ears.

Erythromelalgia is a rare syndrome that is characterized by episodic attacks of burning pain, erythema, and increased temperature usually affecting the extremities, which is aggravated by warmth or exercise. We describe a patient with a 3-year history of refractory burning pain and red ears. A review of clinical features, disease classification, associated diseases, and treatment of this disease is presented.

Biopsy↗
PubMed1999

Skin perfusion in patients with erythromelalgia.

BACKGROUND: Erythromelalgia (EM) is a chronic disorder characterized by intermittent pain, warmth and erythema of the extremities. Symptoms can be precipitated by increasing the temperature of the affected limb and can be partially relieved by direct cooling. MATERIALS AND METHODS: Microvascular assessment was conducted under 'hot' (28 degrees C) environmental conditions in 61 EM (EMI) patients and 30 control subjects. Twenty patients with many of the symptoms of EM were enrolled as an active control group (EMII). Using laser Doppler flowmetry, basal skin erythrocyte flux (SkEF) and the hyperaemic response to local heating (44 degrees C) were measured. RESULTS: Compared with control subjects, basal SkEF was reduced at the toe (P < 0.001), index finger (P < 0.05), dorsal and plantar aspects of the foot (P < 0.01) in both patient groups and at the medial mid-calf (P < 0.05) in EMI patients. Both EM groups also had a significantly reduced maximum SkEF at the dorsum of foot and medial mid-calf (all P < 0.001) compared with control values. In a subset of patients and control subjects, transcutaneous carbon dioxide levels were raised in EMI patients (P < 0.02) compared with levels in control subjects. Toe temperature was significantly reduced in both EM groups compared with control subjects (both P < 0. 001). CONCLUSION: Our study indicates for the first time that there is a vasoconstrictor tendency in patients with EM, which may be related to functional or structural changes in skin microvessels. Thus, the previous hypothesis that the pathophysiology of EM relates to vasodilatation is not supported in our patients. We believe that, in EM, vasoconstriction precedes reactive hyperaemia, similar to that seen in Raynaud's phenomenon.

Adult↗
PubMed1997

Erythromelalgia: a clinical study of 87 cases.

We report on aetiological factors, clinical findings and prognosis of 87 patients with erythromelalgia (EM). This is the largest material reported in the western literature. There is a 100% follow up of patients with observation period up to 11 years. There were 61 females and 26 males. About two-thirds of the patients were primary cases and around three-quarters had a chronic condition. The condition was more common in lower than in upper extremities. Over time patients with erythromelalgic syndrome gradually get worse, those with primary and secondary acute EM get better, whilst primary and secondary chronic EM remain stable.

Adult↗
PubMed1997

Erythromelalgia and vascular complications in polycythemia vera.

The vascular complications in patients with polycythemia vera are microvascular circulatory disturbances typical of thrombocythemia including erythromelalgia, peripheral ischemia, atypical cerebral ischemic attacks, and major arterial and venous thrombotic events. These are positively related to hematocrits due to the increased red cell mass and its concomitant increased whole blood viscosity. Phlebotomy does not prevent the aspirin-responsive microcirculatory circulation disturbances in polycythemia vera because thrombocythemia (platelet count > 400 x 10(9)/L) persists. The risk of major vascular ischemic episodes in poorly controlled polycythemia vera at hematocrits between 0.45 and 0.50 is rather high. The risk of vascular complications in polycythemia vera is best controlled by maintaining the hematocrit at less than 0.45 and the platelet count below 400 x 10(9)/L. The microvascular syndrome associated with thrombocythemia in early stage polycythemia vera in remission by phlebotomy is easily and best controlled by low-dose aspirin (50 to 100 mg) or by selective reduction of platelet count to normal with low-dose myelosuppressive agents. The potential leukemogenic myelosuppressive agents busulfan and hydroxyurea and the nonleukemogenic cytosine interferon-alpha have proven to be effective in the control of the proliferative phase of polycythemia vera. However, data on the natural history of polycythemia vera and the best treatment modality of the various stages of myeloproliferative disease are still lacking.

Aspirin↗
PubMed1994

[Cervical epidural infusion of morphine and bupivacaine in severe erythromelalgia].

A 13 year old boy was admitted suffering from severe burning pain in both hands and distal forearms of two months duration. The skin in the affected areas was red and warm and was cooled by the boy with ice for alleviation of pain. Laboratory tests revealed no abnormalities. The diagnosis of primary erythromelalgia was made on the basis of the history and physical examination. At the age of nine months the boy had undergone surgery for hexadactyly of the right hand. In addition, the boy had complained of transient burning sensations in both feet several months prior to admission. Revision of the old scar at the beginning of the hand symptoms had been without effect. Initial analgesic attempts with oral paracetamol, acetylsalicylic acid, tramadol and intravenous morphine also had had no effect. Carbamazepine, methysergide and immunoglobulins led to no improvement. Symptomatic relief was achieved only by cooling the distal parts of both arms with water. This caused oedema and maceration of the skin after several days. The placement of a cervical epidural catheter and the infusion of morphine and bupivacaine led to a pronounced pain relief within hours and the forearms could be removed from the cool water. The epidural infusion was continued for two weeks with slowly decreasing amounts of morphine and bupivacaine. During this time there was a marked improvement in both oedema and erythema. No complications resulted from the cervical epidural infusion. Because of the long-standing immobilisation and water exposure the boy had to undergo intensive training of the upper extremities during the following weeks to regain full muscular function.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2006

Size matters: Erythromelalgia mutation S241T in Nav1.7 alters channel gating.

The Nav1.7 sodium channel is preferentially expressed in most nociceptive dorsal root ganglion neurons and in sympathetic neurons. Inherited erythromelalgia (IEM, also known as erythermalgia), an autosomal dominant neuropathy characterized by burning pain in the extremities in response to mild warmth, has been linked to mutations in Nav1.7. Recently, a substitution of Ser-241 by threonine (S241T) in the domain I S4-S5 linker of Nav1.7 was identified in a family with IEM. To investigate the possible causative role of this mutation in the pathophysiology of IEM, we used whole-cell voltage-clamp analysis to study the effects of S241T on Nav1.7 gating in HEK293 cells. We found a hyperpolarizing shift of activation midpoint by 8.4 mV, an accelerated time to peak, slowing of deactivation, and an increase in the current in response to small, slow depolarizations. Additionally, S241T produced an enhancement of slow inactivation, shifting the midpoint by -12.3 mV. Because serine and threonine have similar biochemical properties, the S241T substitution suggested that the size of the side chain at this position affected channel gating. To test this hypothesis, we investigated the effect of S241A and S241L substitutions on the gating properties of Nav1.7. Although S241A did not alter the properties of the channel, S241L mimicked the effects of S241T. We conclude that the linker between S4 and S5 in domain I of Nav1.7 modulates gating of this channel, and that a larger side chain at position 241 interferes with its gating mechanisms.

Action Potentials↗
PubMed1992

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↗
PubMed1990

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↗
PubMed1994

The use of capsaicin cream in a case of erythromelalgia.

We present a case of erythromelalgia in a 68 year old lady who responded, within 48 hours, to a twice daily topical application of capsaicin cream 0.025%. Capsaicin cream was stopped after 2 months, and 6 months later the patient continued to have the symptomatic relief she experienced initially.

Aged↗
PubMed1996

Erythromelalgia: a clue to the diagnosis of polycythemia vera.

We report the case of a 74-year-old woman with recurrent episodes of symmetrical congestion and erythema in the distal lower legs causing a burning distress. Laboratory and clinical investigations revealed an underlying myeloproliferative disorder. The cutaneous symptoms were atypical of erythromelalgia. Salicylates and treatment of the underlying polycythemia were able to eliminate the skin lesions but not entirely suppress the subjective discomfort.

Aged↗
PubMed1994

Thrombocythemic erythromelalgia, primary erythermalgia, and secondary erythermalgia: three distinct clinicopathologic entities.

On the basis of clinical, laboratory, and histopathologic studies, the authors discern three distinct types of red, congested, and burning extremities that need to be distinguished for effective treatment according to their etiology: erythromelalgia in thrombocythemia, primary erythermalgia, and secondary erythermalgia. Each entry is discussed in turn.

Erythromelalgia↗
PubMed1981

Erythromelalgia-like eruption in parkinsonian patients treated with bromocriptine.

In a previous report about the chronic use of bromocriptine in Parkinson disease, a distinctive cutaneous eruption was noted and termed "erythromelalgia." This eruption has occurred in 9 of 110 patients on chronic bromocriptine therapy; histopathologic examination in 3 patients showed a prominent perivascular lymphocytic infiltration and perivascular edema of the dermis, without frank vasculitis. This is a reversible unwanted effect of bromocriptine therapy.

Aged↗
PubMed1999

Impaired skin vasomotor reflexes in patients with erythromelalgia.

Erythromelalgia (EM) is a chronic disorder characterized by intermittent burning pain, warmth and erythema of the extremities. Increasing the local temperature and dependency of the affected limb(s) precipitates the symptoms, whereas direct cooling and elevation of the limb(s) can provide partial relief. Our previous findings showed that patients with EM have enhanced cutaneous vascular tone at rest and during stimulation, which may be due to an increase in sympathetic neural activity. To test this, we measured skin vasoconstrictor responses to contralateral arm cold challenge (CC) and inspiratory gasp (IG) using laser Doppler flowmetry at the toe pulp and fingertip. These areas were chosen because of their dense sympathetic innervation. An index of the vasoconstrictor response (between 0 and 1) was calculated from the change in skin perfusion from baseline following CC and IG. In control subjects, vasoconstrictor responses to CC at the toe and fingertip were both 0. 70+/-0.02 (mean+/-S.E.M.), which were significantly greater (P<0. 001) than corresponding values in patients with EM (0.37+/-0.04 and 0.45+/-0.04 respectively). Similarly, vasoconstrictor responses to IG were significantly greater (P<0.001) at the toe and fingertip in control subjects (0.70+/-0.03 and 0.70+/-0.02 respectively) compared with values in EM patients (0.27+/-0.03 and 0.45+/-0.15 respectively). These data show that, in contrast with control subjects, patients with EM have diminished sympathetic vasoconstrictor responses to both CC and IG. Denervation supersensitivity may play a part by increasing vasoconstrictor responses to circulating catecholamines, leading to a reduction in skin blood flow. Therefore an interplay between neural and vasoactive agents may be involved in the pathophysiology of EM.

Adult↗
PubMed2002

[Successful intravenous administration of low dose ketamine for pain caused by erythromelalgia: report of a case].

A 15-year-old female complained of reddening, edema, and pain in her hands and feet. The symptoms were relieved upon cooling. From these findings, a diagnosis of erythromelalgia was made. Because none of the oral medication prescribed by dermatologist was effective, the patient was consulted to our department. A low dose of ketamine, a drug considered to be effective for intractable pain, was administered intravenously and the pain subsided significantly. Furthermore, the pain became completely controllable with a combination of intramuscular ketamine injection and other oral medication.

Adolescent↗
PubMed1990

[A study of erythromelalgia in relation to the autonomic nervous system. Report of 321 cases of functional disorders of the autonomic nervous system].

A great majority of erythromelalgia were seen among 321 cases of disorders of the autonomic nervous system. When the function of the autonomic nervous system was disturbed, the acromelic blood vessels under the regulation by the autonomic nervous system manifested themselves in the manner of acroerythema, hot sensation, local swelling and distension, and severe acroesthesia.

Adolescent↗