Phakomatosis pigmentovascularis IIb with hypoplasia of the inferior vena cava and the right iliac and femoral veins causing recalcitrant stasis leg ulcers

J Am Acad Dermatol. 2003 Aug;49(2 Suppl Case Reports):S167-9. doi: 10.1067/mjd.2003.160.

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.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / diagnostic imaging*
  • Adult
  • Femoral Vein / diagnostic imaging
  • Femoral Vein / pathology*
  • Follow-Up Studies
  • Humans
  • Iliac Vein / diagnostic imaging
  • Iliac Vein / pathology*
  • Leg Ulcer / diagnosis
  • Leg Ulcer / etiology*
  • Leg Ulcer / surgery
  • Male
  • Neurofibromatosis 2 / complications
  • Neurofibromatosis 2 / diagnosis*
  • Phlebography / methods
  • Recurrence
  • Risk Assessment
  • Severity of Illness Index
  • Skin Transplantation / methods
  • Treatment Outcome
  • Vascular Diseases / complications
  • Vascular Diseases / diagnosis
  • Vascular Diseases / diagnostic imaging*
  • Vena Cava, Inferior / diagnostic imaging
  • Vena Cava, Inferior / pathology*