title: Phakomatosis pigmentovascularis IIb with hypoplasia of the inferior vena cava and the right iliac and femoral veins causing recalcitrant stasis leg ulcers.
abstract: We present a patient with phakomatosis pigmentovascularis (PPV) type IIb accompanied with venous hypoplasia, from the inferior vena cava to the superficial femoral vein. Although it is not obvious whether this vascular anomaly, which is probably congenital, is coincidental or not, it is possible that the 2 diseases have some relationship each other, because PPV is thought to result from abnormal vasomotor activity during the embryonic period. In the diagnosis of type II PPV, careful examination and several studies are required to determine systemic involvement that may include large vessel changes.
authors.affiliation: Department of Dermatology, St Paul's Hospital, College of Medicine, The Catholic University of Korea, 620-56 Junnong2-Dong, Dongdaemun-ku, Seoul 130-709, Korea.
authors.forename: Jong-Gap
authors.initials: JG
authors.lastname: Park
authors.forename: Kee-Young
authors.initials: KY
authors.lastname: Roh
authors.forename: Hyun-Jeong
authors.initials: HJ
authors.lastname: Lee
authors.forename: Seog-Jun
authors.initials: SJ
authors.lastname: Ha
authors.forename: Jun-Young
authors.initials: JY
authors.lastname: Lee
authors.forename: Sang Seob
authors.initials: SS
authors.lastname: Yun
authors.forename: Keun Woo
authors.initials: KW
authors.lastname: Lim
authors.forename: Kyung Sup
authors.initials: KS
authors.lastname: Song
authors.forename: Jin-Wou
authors.initials: JW
authors.lastname: Kim
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D000328:Adult; D005268:Femoral Vein / Q000000981:diagnostic imaging / Q000473:pathology*; D005500:Follow-Up Studies; D006801:Humans; D007084:Iliac Vein / Q000000981:diagnostic imaging / Q000473:pathology*; D007871:Leg Ulcer / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D008297:Male; D016518:Neurofibromatosis 2 / Q000150:complications / Q000175:diagnosis*; D010690:Phlebography / Q000379:methods; D012008:Recurrence; D018570:Risk Assessment; D012720:Severity of Illness Index; D016038:Skin Transplantation / Q000379:methods; D016896:Treatment Outcome; D014652:Vascular Diseases / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging*; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging / Q000473:pathology*