Phakomatosis pigmentovascularis IIb with hypoplasia of the inferior vena cava and the right iliac and femoral veins causing recalcitrant stasis leg ulcers.
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.
Park
Jong-Gap
JG
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.
Roh
Kee-Young
KY
Lee
Hyun-Jeong
HJ
Ha
Seog-Jun
SJ
Lee
Jun-Young
JY
Yun
Sang Seob
SS
Lim
Keun Woo
KW
Song
Kyung Sup
KS
Kim
Jin-Wou
JW
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*