Abstract：
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.
摘要：
报道了1例色素性血管瘤病（PPV）III型a，以痣的带状疱疹样分布为主要表现。一名41岁的男子出生时，左半身有带状疱疹样分布，即上肢，肩膀和背部。体格检查显示，在左上肢的伸侧以及左肩和背部呈巨大的带状疱疹样分布的浅棕色色素，表面有分散的棕色斑点和不同大小的斑块，这与痣(NS)的出现是一致的。左前肩出现相对较小的暗红色斑块，按压后显示毛细血管扩张和褪色。这与葡萄酒色斑（PWS）的性能一致。皮肤镜检查显示血管和色素斑的外观清晰，局部融合。他没有系统性的参与。该患者被诊断为色素性血管瘤病Ⅲa型。如果需要，NS和PWS可以用激光治疗。
