A Baby with Café-au-lait Macules and Aberrant Mongolian Spot: Report of Phakomatosis Pigmentopigmentaria

Abstract

Phacomatosis pigmentovascularis (PPV) is a term used to describe the association of vascular nevi with different pigmentary nevi. There are a very few case reports describing the association of two pigmented nevi or pigmented anomalies of nevoid character. The pathogenetic mechanism of such association is hypothesized to be twin spotting. Wolf at al has coined the term phakomatosis pigmentopigmentaria for describing the association different pigmenatry nevi or nevoid disorders. We report a rare case of baby born with co-localized segmental CALMs with aberrant Mongolian spots on the trunk. Our case can be considered as an example of this term phakomatosis pigmentopigmentaria.

Keywords

• cafe-au-lait macules
• mongolian spots
• phacomatosis