Port-wine-stain with rare associations in two cases from Kuwait: Phakomatosis pigmentovascularis redefined

The association of a vascular lesion with melanocytic nevi was first described by Ota et, al in 1947, and given the name phakomatosis pigmentovascularis(PPV). Later on this term was used for the cases with simultaneous occurrence of congenital vascular and pigmented (epidermal or melanocytic) anomalies. Our first case is a 12-year-old Kuwaiti boy who had extensive port-wine stain (nevus flammeus) on his left upper limb and left side of the chest, and Becker’s nevus (BN) on the right shoulder. This is the first report of PPV from Kuwait, and it illustrates that although rare PPV may be prevalent worldwide but an under-reported entity. Our second case is a 31-year-old Kuwaiti female with a congenital port-wine stain on left side of the face along with Neurofibromatosis Type I. To the best of our knowledge this association has not been described in the literature so far. To accommodate such cases in the PPV group, we have suggested a modified classification of PPV. Phakomatosis pigmentovascularis