Vascular malformation and common keratinocytic nevus of the soft type: phacomatosis pigmentovascularis revisited.

André Laureano,Isabel Freitas,Jorge Cardoso,Cristina Amaro,Rodrigo Carvalho

doi:10.1155/2014/437085

Abstract

Phacomatosis pigmentovascularis is a rare syndrome characterized by the coexistence of a pigmented nevus and a cutaneous vascular malformation. We report a 5-year-old boy with all the typical findings of phacomatosis pigmentovascularis type Ia. Although its existence according to the traditional classification has been questioned, this case represents a very rare association of a capillary vascular malformation and a common keratinocytic nevus of the soft type.

Highlights

Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by the coexistence of a pigmented nevus and a cutaneous vascular malformation
Histopathological examination of the verrucous plaque showed acanthosis with epidermal papillomatosis and hyperkeratosis (Figure 3). The combination of these findings favoured the diagnosis of a capillary vascular malformation (CVM), nevus flammeus type, and a common keratinocytic nevus of the soft type
PPV was first described by Ota in 1947

Summary

Introduction

Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by the coexistence of a pigmented nevus and a cutaneous vascular malformation. The diagnosis of such syndrome is primarily clinical and further classification is based on the type of pigmented nevus [1]

Case Presentation

Discussion