Cases reported • Erythromelalgia; Erythermalgia

1/5. Coexistence of erythromelalgia and Raynaud's phenomenon.

erythromelalgia is characterized by spontaneous recurrent episodes of redness, heat, and pain of the extremities that can be triggered or worsened by heat. Raynaud's phenomenon occurs in response to cold exposure and presents as pallor of the fingers or toes, often followed by cyanosis and rubor. Although the 2 conditions may appear to be opposites in symptomatology and clinical presentation, there are very rare reports of their coexistence. A case of coexistent erythromelalgia and Raynaud's phenomenon is presented. The pathophysiology is reviewed to elucidate a common mechanism underlying some cases of the 2 seemingly opposite conditions. A review of the literature indicates that causative and pathophysiologic similarities between the 2 conditions may exist in some cases. Rare reports of coexistence of the 2 disease processes further strengthen such research findings.

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2/5. Thalamic stimulation as a treatment for primary erythromelalgia: technical case report.

OBJECTIVE AND IMPORTANCE: We report the use of bilateral thalamic stimulation in a case of primary erythromelalgia with immediate and important pain relief for 3 years. CLINICAL PRESENTATION: A 12-year-old boy experiencing primary erythromelalgia had a 4-year history of recurrent attacks of severe burning pain in both feet, accompanied by local reddening, swelling, and heating of the skin. The attacks were triggered by warmth and exercise. The pain was relieved only by elevation and cooling of the lower limbs, which he achieved by immersing his legs in a bucket of ice water, resulting in severe ulceration of the skin. INTERVENTION: Because of the gradual aggravation of the signs and symptoms and resistance of the patient's condition to several medical therapies, the patient received spinal cord stimulation. The implants were removed twice because of recurrent infection. Finally, the patient was treated with bilateral electrical stimulation of the ventral posterolateral thalamic nucleus, which resulted in important pain control until 3 years later. The patient was able to avoid water immersions, and all ulcerations disappeared. CONCLUSION: We conclude that thalamic stimulation was successful in this case of primary erythromelalgia.

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3/5. 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.

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4/5. erythromelalgia.

erythromelalgia is an extraordinary disorder of unknown etiology and pathophysiology that resembles the post-traumatic reflex dystrophy syndromes but has not been described previously in the orthopedic literature. Its distinctive triad of intense burning extremity pain associated with erythema and increased skin temperature are diagnostic. Primary or idiopathic and a secondary or associated form have been identified. The latter occurs in association with an underlying disease process, especially myeloproliferative disorders. Treatment with pharmacologic agents and surgery are ineffective except in the secondary group where treatment of the associated disorder generally results in a remission. Symptoms in the primary group can be minimized by appropriate environmental control with cooling and avoiding heat-producing situations that would raise skin temperature above a critical thermal threshold.

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5/5. erythromelalgia--a case study.

erythromelalgia is a rare syndrome characterized by pain, redness, and heat involving the lower and, less frequently, the upper extremities. Symptoms occur with local or environmental stimulation and may be mild for years or become disablingly severe. A finding of relief with ice-water immersion helps distinguish erythromelalgia from disorders such as causalgia and reflex sympathetic dystrophy. erythromelalgia may be classified in three ways: (1) early-onset, (2) adult-onset aspirin-sensitive, and (3) adult-onset non-aspirin-sensitive. Treatment options, which include medication, sympathetic blocks, and surgery, are determined by classification.

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