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Disorder characterized by vasodilatation associated with paroxysmal, intense burning pain and episodic reddening of the extremities (mainly feet). The symptoms of redness, heat, pain, and swelling, when not associated with an organic disease, constitute the primary form, which has also been termed “erythermalgia” because of the significance of the heat. The secondary or acquired form is related to underlying medical conditions.

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Acromelalgia; Erythermalgia; Gerhardt Disease; Weir-Mitchell Disease.

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The first case was reported by the Irish physician Richard James Graves in 1834. The American physician Silas Weir Mitchell suggested the term “Erythromelalgia” in 1878, and the German physician Carl Jakob Gerhardt provided further insights into erythromelalgia in 1892.

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Approximately 0.25:100,000 in the general population.

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Autosomal dominant inheritance for primary erythromelalgia susceptibility, with the responsible gene located on chromosome 2q31-32.

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Pathogenesis unknown. Proposed mechanisms involve abnormalities in platelet aggregation, with contributing factors such as vessel wall damage, changes in blood flow patterns, and disturbances in prostaglandin metabolism. Intravascular platelet aggregation appears to play a major role. Endothelial cells in affected areas appear swollen with enlarged nuclei. Smooth muscle cell proliferation and vacuolization of the cytoplasm in the tunica media of the vessels and deposition of interstitial collagen lead to narrowing of the vessels lumen. The internal elastic lamina seems to be split between the proliferated smooth muscle cells resulting in a picture of fibromuscular intimal proliferation, which is (with or without occlusive intravascular thrombosis) a specific erythromelalgia finding. Arterioles are frequently occluded by thrombi rich in platelets or which become completely fibrotic in the presence of peripheral necrosis. Immunofluorescence findings have failed to indicate a specific inflammatory process. It seems therefore that erythromelalgia is not a separate disease entity but rather a pathophysiologic response (microvascular arteriovenous shunting) of the skin microcirculation, with shunting induced by opening of anatomical arteriovenous anastomosis normally present in hands, feet, nose, and ears, or by angioneogenesis.

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Primary and secondary/acquired types occurred. The primary type occurs in children, especially boys, whereas the secondary form is found almost entirely in adults who have an underlying disease (typically polycythemia vera, essential thrombocythemia, chronic myelogenous leukemia, and idiopathic myelofibrosis and other forms of myeloproliferative disorders) but may also include connective tissue disorders, autoimmune collagen vascular diseases (e.g., rheumatoid arthritis, Systemic Lupus Erythematosus (SLE)), infectious diseases (e.g., AIDS), neurologic diseases (e.g., multiple sclerosis, neuropathies), cardiovascular disorders (e.g., arterial hypertension, arteriosclerosis), diabetes mellitus, and drugs (e.g., iodine contrast injection, vaccinations, calcium antagonists). The clinical appearance of both primary and secondary forms is basically identical. The pathognomonic diagnostic criterion of secondary erythromelalgia is rapid pain relief that lasts a few days after one low dose of aspirin, which irreversibly inhibits platelet cyclooxygenase activity. Heat intolerance (and aggravation of symptoms with standing or exercise) and relief of symptoms with cooling (and elevation of the affected body part and rest) are hallmarks of erythromelalgia, whereas warmth exposure not only triggers flaring ...

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