Table 1. Classification of phakomatosis pigmentovascularis.
Type | Vascular feature | Pigmentary feature |
---|---|---|
I | Nevus flammeus | Nevus pigmentosus et verrucous |
II | Nevus flammeus±anemic nevus | Mongolian spot |
III | Nevus flammeus±anemic nevus | Nevus spilus |
IV | Nevus flammeus±anemic nevus | Mongolian spot, nevus spilus |
V | Cutis marmorata telangiectatica congenita | Mongolian spot |
All types are subdivided into type (a) or (b). (a): cutaneous disease only, (b): cutaneous and systemic disease.