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Parkes Weber syndrome occurring in a family with capillary malformations

Brunetti-Pierri, Nicolaa; Seidel, Glen F.b; Levy, Moise L.c; Reid Sutton, V.a

doi: 10.1097/MCD.0b013e3280f6cff2
ORIGINAL ARTICLES
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Parkes Weber syndrome is a disorder characterized by cutaneous blush, arteriovenous fistula, and overgrowth of the affected limb. It has been differentiated from Klippel–Trenaunay syndrome on the basis of the presence of arteriovenous fistula that are always absent in the latter. We report a case of Parkes Weber syndrome with diffuse arteriovenous high flow leading to hemodynamic complications but without radiographic evidence of arteriovenous fistula. There are multiple individuals in the family with capillary malformations inherited in an autosomal dominant pattern. These observations reinforce the suggestions that Parkes Weber syndrome and capillary malformations may share a common pathogenetic pathway.

Departments of aMolecular and Human Genetics

bRadiology

cDermatology, Baylor College of Medicine, Houston, Texas, USA

Correspondence to V. Reid Sutton, MD, Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, BCM225, USA

Tel: +1 832 822 4280; fax: +1 832 825 4294;

e-mail: vsutton@bcm.edu

Received 30 September 2006 Accepted 8 February 2007

© 2007 Lippincott Williams & Wilkins, Inc.