Severe Rhabdomyolysis in Phacomatosis Pigmentovascularis Type IIb associated with Sturge-Weber Syndrome
10.4266/kjccm.2015.30.4.329
- Author:
Bongjin LEE
1
;
Hyung Joo JEONG
;
Yu Hyeon CHOI
;
Chong Won CHOI
;
June Dong PARK
Author Information
1. Department of Pediatrics, Seoul National University Hospital, Seoul, Korea. jdparkmd@snu.ac.kr
- Publication Type:Case Report
- Keywords:
intermittent claudication;
intracranial aneurysm;
phacomatosis pigmentovascularis;
renovascular hypertension;
rhabdomyolysis;
Sturge-Weber syndrome;
vascular disease
- MeSH:
Arteries;
Constriction, Pathologic;
Humans;
Hypertension;
Hypertension, Renovascular;
Intermittent Claudication;
Intracranial Aneurysm;
Neurocutaneous Syndromes;
Nevus;
Port-Wine Stain;
Rhabdomyolysis;
Sturge-Weber Syndrome;
Vascular Diseases
- From:The Korean Journal of Critical Care Medicine
2015;30(4):329-335
- CountryRepublic 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.
