Erythromelalgia as a presenting manifestation in a patient with essential thrombocythemia complicating renovascular hypertension due to unilateral renal artery stenosis.
Erythromelalgia is a kind of cutaneous manifestation, which appears as a thrombotic complication in patients with myeloproliferative disorders such as essential thrombocythemia and polycythemia vera. It is characterized by red, congested distal extremities and a painful burning sensation, and is usually confined to the feet and one or more toes or fingers. A 28-year-old woman visited our hospital due to severe pain in the left thumb, index fingers and right toes. Her right toes and left thumb were erythematous, congested, and warm. She had a high blood pressure level of 190/100 mmHg, and laboratory evaluation revealed marked thrombocytosis. Bone-marrow findings were compatible with essential thrombocythemia. Renal angiography showed obvious stenosis in unilateral right renal artery. Her erythromelalgia immediately disappeared following interventional therapy along with aspirin. A careful history and appropriate evaluation of underlying diseases are important, because erythromelalgia as a microscopic thrombotic complication may be accompanied by vascular stenosis and all the resulting manifestations.
Hur
Jin-Wuk
JW
Division of Rheumatology, Department of Internal Medicine, The Hospital for Rheumatic Diseases, Hanyang University, 17 Haengdang-Dong, Seoul, 133-792, Korea.
Lee
Young-Yiul
YY
Lee
Woong-Soo
WS
Jun
Jae-Bum
JB
D000328:Adult; D000792:Angiography / Q000379:methods; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D000077144:Clopidogrel; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D004916:Erythromelalgia / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D005260:Female; D006801:Humans; D006978:Hypertension, Renovascular / Q000150:complications / Q000175:diagnosis*; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D012077:Renal Artery / Q000473:pathology; D012078:Renal Artery Obstruction / Q000150:complications / Q000175:diagnosis*; D013922:Thrombocytosis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D013988:Ticlopidine / Q000031:analogs & derivatives / Q000627:therapeutic use; D016896:Treatment Outcome
D000894:Anti-Inflammatory Agents, Non-Steroidal; D010975:Platelet Aggregation Inhibitors; D000077144:Clopidogrel; D013988:Ticlopidine; D001241:Aspirin