Korean J Crit Care Med.  2015 Nov;30(4):329-335. 10.4266/kjccm.2015.30.4.329.

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

• Lee B
• Jeong HJ
• Choi YH
• Choi CW
• Park JD

• KMID: 2156190
• DOI: http://doi.org/10.4266/kjccm.2015.30.4.329

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.

Keyword

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

MeSH Terms

Arteries
Constriction, Pathologic
Humans
Hypertension
Hypertension, Renovascular
Intermittent Claudication
Intracranial Aneurysm
Neurocutaneous Syndromes*
Nevus
Port-Wine Stain
Rhabdomyolysis*
Sturge-Weber Syndrome*
Vascular Diseases

Figure

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  Fig. 1. Brain magnetic resonance imaging shows diffuse leptomeningeal enhancement in the right temporoparietooccipital lobes and an enlarged right choroid plexus (A); an expansile soft tissue mass with heterogeneous signal intensity and peripheral enhancement in the right maxillary sinus (B).

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  Fig. 2. Nevus of Ota and nevus flammeus on the faces (A); nevus flammeus distributed over the abdomen (B); widespread nevus flammeus involving the upper (C) and lower (D) extremities.

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  Fig. 3. Brain magnetic resonance imaging arteriography reveals an aneurysm in the left posterior communicating artery (arrow).

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  Fig. 4. Brain computed tomography shows a focal high attenuated lesion in the pons.

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  Fig. 5. Computed tomography angiography of the lower extremities shows diffuse narrowing of the arteries (A); follow-up imaging obtained on day 17 of admission showing improvement in the previously noted diffuse arterial narrowing (B).

Reference

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