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Severe Rhabdomyolysis in Phacomatosis Pigmentovascularis Type IIb associated with Sturge-Weber Syndrome

Yu Hyeon Choi|이봉진|Hyung Joo Jeong|Chong Won Choi|June Dong Park
대한중환자의학회 (The Korean Society Of Critical Care Medicine)|2015.11|Acute and Critical Care|제30권 제4호|pp. 329-335

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참고문헌

키워드

rhabdomyolysisSturge-Weber syndromevascular diseaseintracranial aneurysmintermittent claudicationphacomatosis pigmentovascularisrenovascular hypertension

초록

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.

Abstract

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.

참고문헌 (20)

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