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혈관염 형태로 나타난 12세 여아의 홍색 사지통증

혈관염 형태로 나타난 12세 여아의 홍색 사지통증

Erythromelalgia in a 12-year old girl with vasculitis feature

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Release Date : 2013. 10. 18(금)
Mo Kyung Jung, Lucy Youngmin Eun, Jo won Jung, Jae Young Choi, Kyu Earn Kim
Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
정모경, 은영민, 정조원, 최재영, 김규언
연세대학교 의과대학 소아과학교실

Abstract

Erythromelalgia is a rare clinical syndrome characterized by erythema, increased temperature, and severe burning pain which can be aggravated by warmth or can be relieved by cold. Erythromelalgia occurs either as a primary, idiopathic form or secondary form to a number of disease and conditions. Although fairly well studied in adults, the characteristics, pathogenesis, natural history are poorly characterized in the pediatric age group. Different therapeutic options have been tried, but no optimal treatment has been suggested for erythromelalgia.We report a rare case of erythromelalgia in a 12-year old girl involving the central body with vasculitis feature. It seems to be a secondary due to vasculitis, but it is uncertained. The erythema was visible as red lines that seemed to follow her vessels and the pain was progressive which was sensitive to heat. Her left upper arm was first, then the pain appeared to cross her torso and afflict her right arm as well, finally resulting in her complaining of bilateral numbness in her hands and feet. We started with a regimen of dexamethasone for 3 days, followed by prednisone, aspirin and propranolol. However, the pain had crossed her torso and afflicted both her legs and then she complained heat sensation whenever she urinated, accompanied by vasc

Keywords: Erythromelalgia, Vasculitis, Pediatrics