Phakomatosis pigmentovascularis type IVa

Phakomatosis pigmentovascularis was first reported in 1947. We describe a 1-year-old Japanese girl who, since birth, has had three nevoid skin disorders: nevus flammeus, nevus spilus, and aberrant mongolian spots. No systemic disease of any kind has been present. We believe that this phakomatosis, which we have named phakomatosis pigmentovascularis type IVa, does not reflect the merely coincidental coexistence of three kinds of nevi.