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. 2017 Aug 25;29(5):638–639. doi: 10.5021/ad.2017.29.5.638

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.