Phakomatosis pigmentovascularis type Ⅲa mainly manifested by zosteriform nevus spilus: A case report with dermoscopic features.

A case of phakomatosis pigmentovascularis (PPV) type III a with a zosteriform distribution of nevus spilus as the main manifestation was reported. A 41-year-old man was born with a zosteriform distribution of pigmented rash on the left half-body, namely upper limb, shoulder and back. Physical examination revealed light brown pigments in a giant zosteriform distribution on the extensor side of the left upper limb and the left shoulder and back, with scattered brown spots and patches of variant sizes on the surface, which are consistent with the appearance of nevus spilus (NS). A relatively small area of dark red patch occurred on the left anterior shoulder, which showed telangiectasia and fading after pressing. It was consistent with the performance of a port-wine stain (PWS). Dermoscopy showed a clearer appearance and localized fusion of blood vessels and pigmented patch. He has no systemic involvement. The patient was diagnosed with phakomatosis pigmentovascularis type Ⅲa. NS and PWS can be treated with lasers if required.