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

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

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...

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Bibliographic Details
Main Authors: Bongjin Lee, Hyung Joo Jeong, Yu Hyeon Choi, Chong Won Choi, June Dong Park
Format: Article
Language:English
Published: Korean Society of Critical Care Medicine 2015-11-01
Series:Korean Journal of Critical Care Medicine
Subjects:
intermittent claudication
intracranial aneurysm
phacomatosis pigmentovascularis
renovascular hypertension
rhabdomyolysis
Sturge-Weber syndrome
vascular disease
Online Access:http://kjccm.org/upload/pdf/kjccm-2015-30-4-329.pdf
Description
Summary: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.
ISSN:2383-4870
2383-4889

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