Hematoporphyrin monomethyl ether-mediated photodynamic therapy for phakomatosis pigmentovascularis type II: A case report

Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized by capillary malformation (mainly port-wine stains, PWS) and pigmentary nevi with or without extracutaneous signs. Efforts are ongoing to develop laser therapy to treat vascular and pigmentation abnormalities in PPV. The status of pulsed-dye lasers in the treatment of PWS has been challenged by vascular-targeted photodynamic therapy (PDT). Hematoporphyrin monomethyl ether-mediated PDT (HMME-PDT) has become a research hotspot for PWS. The long-term efficiency and safety of HMME-PDT for vascular lesions coexisting with pigmented lesions in PPV have not been reported. We report a pediatric case of PPV type IIa presenting with PWS and nevus of Ota efficiently treated through a 3 session HMME-PDT. After treatment, the PWS lesions regressed significantly without noticeable adverse reactions or recurrence. Besides, we share the dermoscopy data of PPV lesions and discuss the clinical manifestations and therapeutic strategies for PPV based on existing related literature.