Thrombocythemia as a cause of erythermalgia

Abstract

Erythermalgia is a distinctive clinical entity characterized by local redness, warmth, and burning pain in the distal portions of the extremities. In this study, erythemalgia was observed in two patients with myeloproliferative disease. A new theory suggesting abnormally rapid platelet aggregation as a cause of erythermalgia was advanced. The following evidence is presented: (1) histologic evidence of thrombi occluding the arterioles and probably composed of platelet aggregates; (2) reversal of the clinical findings with platelet aggregating drugs, such as heparin sodium and acetylsalicyclic acid; (3) disappearance of the erythemalgia with the adequate control of the myeloproliferative disease.

Paraneoplastic vasculitis and paraneoplastic vascular syndromes

“…About 20% of cases are associated to hematological malignancies, such as thrombocythemia or polycythemia (38), and the average span between disease occurrence and the diagnosis of neoplasm is estimated in 2.5 years. In these secondary cases, it is thought that platelet breakdown products and platelet‐rich microthrombi may be the basis of the disease (39).…”

Erythromelalgia: A Pathognomonic Microvascular Thrombotic Complication in Essential Thrombocythemia and Polycythemia Vera

“…Interestingly, there was no evidence of arteriosclerosis. In 1977, Redding 10 described a patient with ET as a cause of erythromelalgia complicated by ischemic acrocyanosis of the little toe. Punch skin biopsies showed occluding thrombi in many capillaries and in some dermal arteries, arterioles, and venules.…”

Livedo reticularis as a presenting symptom of polycythaemia vera

“…Perhaps the most frequent feature is cyanosis and purple coloration of the face. About 15% of the patients present with pruritus, 1 3% with erythromelalgia 2 . Aquagenic pruritus, 3 urticaria vasculitis, 4 repeated attacks of erythromelalgia 5 were described as initial features of PV.…”