Primary erythromelalgia: a review

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Primary erythromelalgia: a review

Primary erythromelalgia (PE ORPHA90026) is a rare autosomal dominant neuropathy characterized by the combination of recurrent burning pain, warmth and redness of the extremities. The incidence rate of PE ranges from 0.36 to 1.1 per 100,000 persons. Gender ratio differs according to different studies and no evidence showed a gender preference. Clinical onset of PE is often in the first decade of...

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Primary erythromelalgia: case report.

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 disor...

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Dual Effect of Ziconotide in Primary Erythromelalgia

Erythromelalgia (EM) is a rare disabling clinical syndrome more commonly known to affect the lower extremities. There is no single effective treatment for this disease that often requires a multidisciplinary approach. Herein, we report the case of a 31-year-old woman affected by primary erythromelalgia who was successfully treated with intrathecal Ziconotide. We also observed an unexpected resu...

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Treatment of primary erythromelalgia with cyclosporine.

A 28-year-old woman presented with severe, persistent burning pain in the legs and feet, with marked erythema, swelling, and warmth (Fig. 1, left-hand panels). The erythema had begun at the age off our years, with no associated disorder. After adolescence, the symptoms became continuous, with erythema that extended to the thighs and the development of live do-like lesions. Burning pain during t...

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Sympathetic Block for Treating Primary Erythromelalgia

Primary erythromelalgia is a rare condition that's characterized by erythema, an increased skin temperature and burning pain in the extremities. The pain is often very severe, and treating erythromelalgia is frustrating and difficult. We report here on the case of a 12-year old girl with primary erythromelalgia in both lower extremities. The pain was refractory to medical treatment, but a bilat...

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ژورنال

عنوان ژورنال: Orphanet Journal of Rare Diseases

سال: 2015

ISSN: 1750-1172

DOI: 10.1186/s13023-015-0347-1