Phacomatosis Pigmentovascularis is a rare syndrome characterized by capillary malformation and pigmentary nevus. A case of a 2-year-old patient is reported, who presented extensive nevus flammeus and an aberrant Mongolian spot, without systemic disease, manifestations that allow us to classify this case as type IIa Phacomatosis Pigmentovascularis, according to Hasegawa's classification.
Hemangioma; Nevus of Ota; Nevus, pigmented
| Type | Type | Vascular lesion | Pigmentary lesion |
| (according to Happle) | (according to Hasegawa* * All subtypes, according to Hasegawa, are subdivided in types (a) or (b) - with and without systemic involvement, respectively ) | ||
| (inexistent** ** According to Happle, the subtype I does not exist for there are no reported cases to this day ) | I | Nevus flammeus | Verrucous and pigmented nevus |
| Cesioflammea | II | Nevus flammeus ± anemic nevus | Mongolian spot |
| Spilorosea | III | Nevus flammeus ± anemic nevus | Nevus Spilus |
| Non-classifiable | IV | Nevus flammeus ± anemic nevus | Mongolian spot, Nevus Spilus |
| Cesiomarmorata | V | Cutis Marmorata Telangiectatica Congenita | Mongolian spot |
