Department of Dermatology, First Affiliated Hospital of Anhui Medical University, 218 Jixi Road, Shushan District, Hefei, Anhui 230022, China; Key Laboratory of Dermatology, (Anhui Medical University), Ministry of Education, Hefei, Anhui 230022, China; Inflammation and Immune-Mediated Diseases Laboratory of Anhui Province, Hefei, Anhui 230022, China.
Department of Dermatology, First Affiliated Hospital of Anhui Medical University, 218 Jixi Road, Shushan District, Hefei, Anhui 230022, China.
Photodiagnosis Photodyn Ther. 2022 Mar;37:102637. doi: 10.1016/j.pdpdt.2021.102637. Epub 2021 Nov 18.
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.
色素血管性错构瘤病（PPV）是一种罕见的先天性综合征，其特征为毛细血管畸形（主要为葡萄酒色斑，PWS）和色素痣，伴有或不伴有皮肤外表现。目前正在努力开发激光疗法以治疗 PPV 中的血管和色素异常。血管靶向光动力疗法（PDT）对脉冲染料激光治疗 PWS 的地位提出了挑战。血卟啉单甲醚介导的 PDT（HMME-PDT）已成为 PWS 的研究热点。PPV 中同时存在血管病变和色素病变时，HMME-PDT 的长期疗效和安全性尚未见报道。我们报告了一例 2a 型 PPV 患儿，经 3 次 HMME-PDT 治疗 PWS 和太田痣，疗效显著。治疗后，PWS 病变明显消退，无明显不良反应或复发。此外，我们还分享了 PPV 病变的皮肤镜数据，并根据现有相关文献讨论了 PPV 的临床表现和治疗策略。
