Phacomatosis pigmentovascularis Type IIa – A case report

Authors

  • Meenakshi Girish
  • G Nanoti
  • Y Banait

DOI:

https://doi.org/10.32677/IJCH.2016.v03.i02.023

Keywords:

Mongolian spots, Nevus flammeus, Vascular malformations

Abstract

Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by capillary vascular malformation and pigmentary nevus but with a wide variability in clinical presentation. A case of a 9-month-old patient is reported, who presented with capillary malformation and hemihypertrophy. These features typically are seen in Klippel–Trenaunay (KT) syndrome, a syndromic type of congenital vascular malformation. However, in addition, this child had large persistent Mongolian spots, the presence of which allowed us to classify this case as PPV, type II. This is a condition with a clinical course not always benign as KT syndrome.

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Published

2016-06-27

Issue

Section

Case Reports

How to Cite

Phacomatosis pigmentovascularis Type IIa – A case report. (2016). Indian Journal of Child Health, 3(2), 181-182. https://doi.org/10.32677/IJCH.2016.v03.i02.023

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