Mapo-Gongdeok S&U Dermatology Clinic, Seoul, Korea.
Department of Dermatology, SMG-SNU Boramae Medical Center, Seoul, Korea.
J Dermatol. 2019 Oct;46(10):843-848. doi: 10.1111/1346-8138.15035. Epub 2019 Aug 6.
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.
色素血管性错构瘤病是一种罕见的综合征，其特征为广泛的毛细血管畸形和色素性痣。本研究旨在评估其特征和治疗方法。回顾性分析了 2003 年至 2017 年间就诊的 52 例患者。最常见的是 IIa 型（葡萄酒色斑和真皮黑素细胞痣伴眼皮肤受累）。全身性受累占 17.3%，与毛细血管畸形或色素痣的范围无明显相关性。然而，眼颧部褐青色痣患者和头颈部色素痣患者的全身性受累明显更为常见（P = 0.004 和 0.035）。脉冲染料激光治疗使 28.6%的患者毛细血管畸形几乎消退，而 Q-开关钕：钇铝石榴石激光治疗使 23.7%的患者色素痣几乎消退，42.1%的患者完全消退。治疗结果评分与葡萄酒色斑或色素痣的范围呈显著负相关（P = 0.047，ρ=-0.308 和 P = 0.011，ρ=-0.410）。色素痣对激光的治疗反应优于毛细血管畸形。无论是毛细血管畸形还是色素痣，较小的病变都倾向于获得更好的治疗效果。
