Phacomatosis pigmentovascularis type IIIb.

Phacomatosis pigmentovascularis (PPV) is a group of syndromes comprising coexistent vascular and pigmentary nevi, which is first classified into five subtypes and then further categorized according to the absence or presence of associated systemic findings as type (a) and (b), respectively. This case report is of an 11-year-old Turkish girl with extensive vascular nevus and nevus spilus. Because of coexistent scoliosis, the patient represents an example of PPV type IIIb. Including the present case, a total of eight cases of PPV type IIIb have been reported to date. The case is discussed in view of the new classification proposed by Happle.