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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Oral healthcare management of a child with phakomatosis pigmentovascularis associated with bilateral Sturge‐Weber syndrome Mariana Leonel Martins, Aline Dos Santos Letieri, Michele Machado Lenzi, Michelle Agostini, Gloria Fernanda Castro Special Care in Dentistry.2019; 39(3): 324. CrossRef