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A 69-year-old woman with complete atrioventricular block was admitted for implantation of a pacemaker. Her medical history included multiple sclerosis with tetraparesis. She had been taking acenocoumarol for the previous 10 years for the treatment of deep venous thrombosis. Twenty-four hours after acenocoumarol was withdrawn before pacemaker implantation, intense lingual pain developed. On physical examination, a well-circumscribed, triangular necrotic area was detected on the tip of the tongue (Panel A). Surgical excision was performed. Histopathological analysis revealed ischemic necrosis and intraluminal thrombi without vasculitis (Panel B, arrow). The total peripheral-blood platelet count was 700,000 per cubic millimeter, and the diagnosis of essential thrombocytosis was confirmed on bone marrow biopsy and detection of the JAK2 V617F mutation. The patient's previous platelet count was unknown. Therapy with acenocoumarol and hydroxyurea was initiated, and the patient had a full recovery. The tongue is a rare location of ischemic necrosis, even in patients with essential thrombocytosis, because of the excellent blood supply and collateral circulation.
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