Korean Journal of Critical Care Medicine  2015;30(4):329-335

doi:10.4266/kjccm.2015.30.4.329

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

Bongjin LEE ; Hyung Joo JEONG ; Yu Hyeon CHOI ; Chong Won CHOI ; June Dong PARK

Keywords

intermittent claudication; intracranial aneurysm; phacomatosis pigmentovascularis; renovascular hypertension; rhabdomyolysis; Sturge-Weber syndrome; vascular disease

Country

Republic of Korea

Language

English

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.