JAN J. MICHIELS, M.D.; ABELS JOHANNES, M.D.; JOHAN STEKETEE, Ph.D.; HUUB H. D. M. VAN VLIET, Ph.D.; VOJISLAV D. VUZEVSKI, M.D.
Erythromelalgia was the presenting symptom in 26 of 40 patients with thrombocythemia in its primary form or when associated with polycythemia vera. The localized painful burning, redness, and warm congestion in the extremities could be accurately documented with thermography. Skin punch biopsy samples taken from the affected areas showed typical arteriolar inflammation, fibromuscular intima proliferation, and thrombotic occlusions. Erythromelalgia often progressed to ischemic acrocyanosis or necrosis in toes or fingers. Complete relief of pain and restoration of microvascular circulation disturbances was obtained with the cyclo oxygenase inhibitors aspirin and indomethacin, but not with sodiumsalicylate or the platelet inhibitors dipyridamole, sulfinpyrazone, ticlopidine, and dazoxiben. The erythromelalgia was alleviated during busulfan-induced remissions of thrombocythemia and its recurrence coincided with relapsing thrombocythemia. These observations suggest a causal relationship between erythromelalgia and thrombocythemia, in which platelet-mediated inflammatory and occlusive arteriolar changes play a part in the etiology of erythromelalgia.
MICHIELS JJ, JOHANNES A, STEKETEE J, et al. Erythromelalgia Caused by Platelet-Mediated Arteriolar Inflammation and Thrombosis in Thrombocythemia. Ann Intern Med. 1985;102:466–471. doi: 10.7326/0003-4819-102-4-466
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Published: Ann Intern Med. 1985;102(4):466-471.
Cardiology, Hematology/Oncology, Platelet Disorders.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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