Clinical characteristics and treatment of 52 cases of phakomatosis pigmentovascularis.

Phakomatosis pigmentovascularis is a rare syndrome characterized by widespread capillary malformation and pigmented nevus. The objective of this study was to evaluate its characteristics and treatment. Fifty-two patients presenting between 2003 and 2017 were retrospectively reviewed. Type IIa (port-wine stain and dermal melanocytosis with oculocutaneous involvement) was most common. Systemic involvement was observed in 17.3% and it was not significantly correlated to extent of capillary malformation or pigmented nevus. However, systemic involvement was significantly frequent in patients with nevus of Ota and in patients with pigmented nevus located on the head and neck (P = 0.004 and 0.035, respectively). Capillary malformation was almost cleared in 28.6% of patients using pulsed dye laser, whereas pigmented nevus was almost cleared in 23.7% and completely cleared in 42.1% of patients using Q-switched neodymium:yttrium-aluminum-garnet laser. Treatment outcome score showed significant inverse correlation with the extent of port-wine stain or pigmented nevus (P = 0.047, ρ = -0.308 and P = 0.011, ρ = -0.410, respectively). Pigmented nevus demonstrated better treatment response to lasers than did capillary malformation. Smaller lesions tended to show better treatment outcomes for both capillary malformation and pigmented nevus.