Phakomatosis pigmentovascularis is a rare neurocutanous condition where there is coexistence of a capillary malformation (port-wine stain) with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota.
Types
Phakomatosis pigmentovascularis is subdivided into five types:
Type 1 PWS + epidermal nevus
Type 2 (most common): PWS + dermal melanocytosis +/- nevus anemicus
Type 3: PWS + nevus spilus +/- nevus anemicus
Type 4: PWS + nevus spilus + dermal melanocytosis +/- nevus anemicus
Type 5: CMTC (Cutis marmorata telangiectatica congenita) + dermal melanocytosisThey all can contain capillary malformation. [Source: Wikipedia 
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