Phacomatosis Pigmentovascularis: A New Syndrome? Report of Four Cases

We examined four patients who had a combination of extensive nevus flammeus, significant oculocutaneous pigmentation, and severe neurologic alterations. All cases were sporadic. The vascular and neurologic alterations were clinicaily similar to those observed in the Sturge-Weber syndrome. The capillary vessels are ultrastructurally different in phacomatosis pigmentovascularis from those of a nevus flammeus in the Sturge-Weber syndrome; however, tnelanocytes of normal aspect were present in the middle and deep der-mis. The characteristic oculocutaneous pigmentation probably represented a noncoincidental association. The term phacomatosis pigtnentovascuiaris seems appropriate for this apparently new neurocutaneous syndrome.