Phakomatosis pigmentovascularis
Phakomatosis pigmentovascularis is a rare neurocutanous condition where there is coexistence of a capillary malformation with various melanocytic lesions, including dermal melanocytosis, nevus spilus, and nevus of Ota.Types
Phakomatosis pigmentovascularis is subdivided into five types:
They all can contain capillary malformation. Type 2 is the most common and can be associated with granular cell tumor. Some further subdivide each type into categories A & B; with A representing oculocutaneous involvement and subtype B representing extra oculocutaneous involvement. Others have proposed fewer subtypes but currently this rare entity is mostly taught as having five subtypes currently.Diagnosis
Treatment
