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
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;
Received 30 September 2006 Accepted 8 February 2007