Microvascular Arteriovenous Shunting is a Probable Pathogenetic Mechanism in Erythromelalgia

Mørk, Cato; Asker, Claes; Salerud, E. Göran; Kvernebo, Knut

doi:10.1046/j.1523-1747.2000.00944.x

Cited by 69 publications

(47 citation statements)

References 25 publications

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“…In a prospective study, Kalgaard et al (15) reported histopathological findings characterized by capillary proliferation or vascular damage in 31 of 49 specimens, mainly from patients with PEM. These nonspecific alterations indicated the presence of skin hypoxia secondary to increased arteriovenous shunting and insufficient capillary flow, which is compatible with the shunting hypothesis supported by Mørk et al (13,14).…”

Section: Discussionsupporting

confidence: 87%

“…According to the shunting hypothesis, EM symptoms are caused by tissue hypoxia, which is induced by a maldistribution of skin microvascular blood flow with increased thermoregulatory flow through arteriovenous shunts and an inadequate nutritive perfusion to normal skin capillaries (13,14). If available blood is shunted away from normal skin capillaries, the skin will be hypoxic.…”

Section: Discussionmentioning

confidence: 99%

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Secondary erythromelalgia successfully treated with patient-controlled epidural analgesia and interferon α-2b: A case report and review of the literature

Li²,

Yao

et al. 2016

Experimental and Therapeutic Medicine

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Abstract. Erythromelalgia (EM) is a debilitating neurovascular disease that is refractory to numerous treatment modalities. The present study reported the case of a 72-year-old woman diagnosed with EM secondary to polycythemia vera (PV), who was effectively treated with the use of patient-controlled epidural analgesia (PCEA) and interferon α-2b therapy. The study aimed to provide clinical information on this condition based on the findings of the present case and previously-reported cases. PCEA containing local anesthetics and dexamethasone or fentanyl resulted in nearly complete relief of symptoms. Furthermore, continuous use of interferon α-2b for the treatment of the PV completely relieved the EM symptoms. In conclusion, PCEA and interferon α-2b may be promising treatments for EM secondary to PV.

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Section: Discussionsupporting

confidence: 87%

Section: Discussionmentioning

confidence: 99%

Secondary erythromelalgia successfully treated with patient-controlled epidural analgesia and interferon α-2b: A case report and review of the literature

Li²,

Yao

et al. 2016

Experimental and Therapeutic Medicine

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“…This confirms the observations of Kalgaard et al that suggest that erythromelalgia is not an isolated disease but that it is a pattern of response of cutaneous microvasculature 8 In a prospective study Davis et al observed that erythromelalgia is associated with a neuropathy of small fibers and primary vasculopathies, characterized by an intermittent increase of blood flow, hypoxia, and possibly shunts, with increase in local cellular metabolism. 9,11 Due to the hypothesis of shunts, substances that alter the distribution of skin blood flow can improve the cutaneous oxygenation and induce symptoms relief. Aspirin, which inhibits platelet aggregation can quickly relief the secondary symptoms of coagulopathy.…”

Section: Discussionmentioning

confidence: 99%

Eritromelalgia primária: relato de caso

Albuquerque¹,

França

Kozmhinsky

et al. 2011

An. Bras. Dermatol.

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Erythromelalgia is a rare clinical syndrome characterized by heat, redness and intermittent pain in the extremities, being most frequent the bilateral development in the lower extremities. Local cooling brings relief to symptoms, while heating, physical exercises and use of stockings/socks intensify the discomfort. This condition can be primary or idiopathic or secondary to haematological disorders and vascular inflammatory and degenerative diseases. It is reported the case of an eighteen-year-old male who presented, at the early age of two, development of the symptoms of erythema ,heat and pain followed by desquamation of hands and feet, in outbreaks, with intervals 4 to 5 years long between the crises. Keywords: Erythema; Erythromelalgia; Hot temperature; Pain Resumo: Eritromelalgia é uma síndrome clínica rara, caracterizada por calor, rubor e dor intermitente nas extremidades, sendo frequente o acometimento bilateral das extremidades inferiores. O resfriamento local provoca alívio dos sintomas, enquanto aquecimento, exercícios físicos e uso de luvas e meias intensificam o desconforto. A desordem pode ser primária ou idiopática, ou secundária a distúrbios hematológicos e doenças vasculares inflamatórias e degenerativas. Relata-se o caso de um jovem de dezoito anos, com início precoce aos dois anos de idade dos sintomas de eritema, calor e dor, seguidos de descamação nas mãos e pés, em surtos, com intervalos longos de 4 a 5 anos entre as crises.

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“…6 Mork et al 7 also showed reduced skin capillary density during attacks of erythromelalgia. Prospective studies of a group of patients with erythromelalgia suggest a vasculopathy with increased shunting and flow and increased local cell metabolism or a smallfiber neuropathy.…”

Section: Discussionmentioning

confidence: 95%

Primary Erythromelalgia in a Child Responding to Intravenous Lidocaine and Oral Mexiletine Treatment

et al. 2005

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ABSTRACT. Erythromelalgia is a rare, chronic, debilitating condition characterized by redness, warmth, and severe burning pain of the distal extremities. The feet are more commonly affected than the hands. Pain is precipitated by increases in temperature and by exercise. Patients often obtain relief by immersing the affected extremity in cold water. The pain is often refractory to treatment. For many patients, multiple pain medications have been useless in achieving complete relief of pain symptoms. Previous reports of erythromelalgia among adolescents indicated prolonged relief of pain with sodium nitroprusside infusions, epidural infusions of local anesthetics, or gabapentin treatment. We present a case of an 11-year-old, white, male child with primary erythromelalgia, whose initial symptoms started in his preschool years and whose childhood was marked by escalating episodes of pain with warmth and redness of his feet, precipitated especially by increases in temperature and by activity. All conventional pain management techniques had failed to relieve our patient of his symptoms, and he obtained some relief only by soaking his affected extremities in ice water. He had experienced minimal benefit from seeing a pain psychologist, who helped him develop techniques to cope with the pain. At the time of presentation, the patient's episodes of pain had increased to 15 to 20 per day, and there was evidence of chronic immersion injury to the skin of his feet. Before his most recent hospitalization, the pain had spread to involve his hands as well. The patient was overwhelmed with anxiety and could not participate in school or social activities at the time of admission. During his current hospitalization, he did show some therapeutic response to sodium nitroprusside infusion, which unfortunately had to be discontinued because of side effects and because his family desired to leave the ICU environment, which was stressful to the patient. He also had some response to lumbar epidural infusion of local anesthetics, which could not be continued because he found the motor blockade that accompanied his analgesia intolerable. However, intravenous lidocaine infusion, with subsequent transition to oral mexiletine therapy, proved very effective in reducing the frequency and severity of the pain episodes. The patient was discharged from the hospital with oral mexiletine therapy and has been monitored at the pain management clinic. He returned to and completed school, attended summer camp, and enjoys an active happy life. He walks without precipitating pain in his feet and sleeps 9 to 10 hours every night. He has needed to soak his feet on only 4 occasions in the 6 months since his discharge from the hospital.

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Microvascular Arteriovenous Shunting is a Probable Pathogenetic Mechanism in Erythromelalgia

Cited by 69 publications

References 25 publications

Secondary erythromelalgia successfully treated with patient-controlled epidural analgesia and interferon α-2b: A case report and review of the literature

Secondary erythromelalgia successfully treated with patient-controlled epidural analgesia and interferon α-2b: A case report and review of the literature

Eritromelalgia primária: relato de caso

Primary Erythromelalgia in a Child Responding to Intravenous Lidocaine and Oral Mexiletine Treatment

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