Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030193 (pain)
 261,466 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Erythromelalgia is characterized by paroxysmal burning pain and redness of the distal extremities; primary and secondary forms exist. Secondary erythromelalgia is most commonly associated with essential thrombocythemia and polycythemia vera, and the pathogenic mechanism is thought to be platelet aggregation. Thrombotic thrombocytopenic purpura is characterized by thrombocytopenia, microangiopathic anemia, renal failure, and fluctuating neurologic signs and symptoms. The pathogenic mechanism suggested for thrombotic thrombocytopenic purpura is diffuse platelet aggregation. We describe a female patient in whom thrombotic thrombocytopenic purpura was associated with erythromelalgia. This has not been previously reported.
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PMID:Erythromelalgia in a patient with thrombotic thrombocytopenic purpura. 161 44

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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PMID:Erythromelalgia and myeloproliferative disorders. 264 12

Erythromelalgia is not a commonly recognized or diagnosed condition that affects the lower extremities. The first reported case was in 1878, when Mitchell suggested the term "erythromelalgia." This condition is characterized by a burning sensation with erythema of the involved extremity. When the extremity is lowered, or heat is applied, the pain is intensified. The application of cold or elevation of the extremity will have the opposite effect of decreasing the pain. Erythromelalgia is classified as primary or idiopathic if there is no accompanying disease process. Secondary erythromelalgia is associated commonly with myeloproliferative syndrome-related thrombocythemia, and is mostly evident in adult onset of the condition. Treatment for adults with erythromelalgia includes a single daily dose of aspirin, but children who have no associated underlying disorder find little to no relief with acetylsalicylic acid.
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PMID:Erythromelalgia: diagnosis and classification. 1038 66

Erythromelalgia is an extraordinary pain syndrome first described by S. Weir Mitchell in 1878. Episodes of severe burning pain in the distal limbs, accompanied by striking redness and warmth of the skin, are precipitated by heat or activity and can be terminated only by cooling the affected part. Primary erythromelalgia is a sporadic or autosomal-dominant hereditary disorder whose symptoms begin in childhood. Secondary erythromelalgia occurs in association with thrombocythemia, collagen-vascular diseases, diabetes mellitus, peripheral neuropathy, and use of certain drugs. Aspirin is effective for patients with thrombocythemia, but most other cases are very resistant to treatment. The pathogenesis of erythromelalgia has remained puzzling, especially the peculiar switch-like manner in which symptoms are turned on by heat and turned off by cold. Following Ochoa's description of the ABC (angry backfiring C nociceptors) syndrome, it seems plausible to regard erythromelalgia as a problem of sensitized skin polymodal C-fiber receptors. C-fiber threshold to activation by heat would be lowered to 32 degrees C to 36 degrees C; activated C fibers would cause vasodilation via axon reflexes with redness, heat, and swelling. Cooling would bring the nociceptors below threshold. Secondary erythromelalgia may result from humoral factors released from platelets or ischemic tissues or from C-fiber injury in some cases of neuropathy, whereas primary erythromelalgia could be due to a mutation of the capsaicin receptor.
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PMID:Hot feet: erythromelalgia and related disorders. 1130 88

Secondary erythromelalgia is a rare disease characterized by burning pain, marked erythema, edema, and hyperthermia of the affected limbs. Secondary erythromelalgia can be associated with various systemic diseases. Here, we describe a patient who developed secondary erythromelalgia involving the ears and concomitant clinical and laboratory, probably, indicating the initial stage of a developing lupus erythematosus.
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PMID:Secondary erythromelalgia involving the ears probably preceding lupus erythematosus. 1797 78

Erythromelalgia is a rare intermittent vascular acrosyndrome characterized by the combination of recurrent burning pain, warmth and redness of the extremities. It is considered in its primary form as an autosomal dominant neuropathy related to mutations of SCN9A, the encoding gene of a voltage-gated sodium channel subtype Nav1.7. Secondary erythromelalgia is associated with myeloproliferative disorders, drugs (bromocriptine, calcium channel blockers), or clinical conditions such as rheumatic diseases or viral infection. Primary familial erythromelalgia include genetics and sporadic forms associated with small fibers neuropathy. Aspirin is a useful treatment of erythromelagia associated with myeloproliferative disorders. Treatment of primary erythromelalgia is difficult, individualized, with sodium channel blockers such as lidocaine, carbamazepine and mexiletine.
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PMID:[Erythromelalgia: Diagnosis and therapeutic approach]. 2763 8