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KMID : 0604020150300040329
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Korean Journal of Critical Care Medicine
2015 Volume.30 No. 4 p.329 ~ p.335
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Severe Rhabdomyolysis in Phacomatosis Pigmentovascularis Type IIb associated with Sturge-Weber Syndrome
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Lee Bong-Jin
Jeong Hyung-Joo
Choi Yu-Hyeon
Choi Chong-Won
Park June-Dong
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Abstract
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Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by concurrent nevus flammeus (capillary malformation) and pigmentary nevus. According to current research, the major pathophysiologic mechanism in PPV is venous dysplasia with resultant compensatory collateral channels and venous hypertension. Arterial involvement is rare. We herein report our experience on renovascular hypertension, intermittent claudication, and severe rhabdomyolysis due to diffuse stenosis of multiple arteries in a patient with PPV type IIb associated with SWS.
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KEYWORD
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intermittent claudication, intracranial aneurysm, phacomatosis pigmentovascularis, renovascular hypertension, rhabdomyolysis, Sturge-Weber syndrome, vascular disease
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