Zhang Yunfeng, Wang Dixin, Zhang Linglin, Wen Long, Zhang Haiyan, Zhang Guolong, Wang Xiuli
Institute of Photomedicine, Shanghai Skin Disease Hospital, Tongji University School of Medicine, 1278 Baode Road, Shanghai, 200443, People's Republic of China.
Institute of Photomedicine, Shanghai Skin Disease Hospital, Tongji University School of Medicine, 1278 Baode Road, Shanghai, 200443, People's Republic of China.
Photodiagnosis Photodyn Ther. 2021 Mar;33:102180. doi: 10.1016/j.pdpdt.2021.102180. Epub 2021 Jan 8.
Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized by (a) capillary malformations, such as port-wine stain (PWS), and (b) pigmented lesions, such as pigmented trichoepidermal nevus, café-au-lait spots, and dermal melanocytosis with or without systemic damage. Severe pruritus has not previously been reported among PPV patients. Here, we report a pediatric case of PPV with severe pruritus, which was refractory to various long-term oral antiallergic agents and topical tacrolimus but was temporarily responsive to hematoporphyrin monomethyl ether-mediated photodynamic therapy (HMME-PDT). HMME-PDT is the latest technology used in treating PWS following the basic principle of targeted photodynamic destruction of the vascular wall of the lesion. Furthermore, many studies have confirmed the safety and efficacy of HMME-PDT for PWS in both adults and children. Specific pathophysiologic mechanisms and treatment methods must be further explored to advance our understanding of the disease and improve the quality of life in PPV patients.
色素血管性斑痣性错构瘤病（PPV）是一种罕见的先天性综合征，其特征为：（a）毛细血管畸形，如葡萄酒色斑（PWS）；（b）色素沉着性病变，如色素性毛发表皮痣、咖啡斑和真皮黑素细胞增多症，伴或不伴有全身损害。此前尚未有PPV患者出现严重瘙痒的报道。在此，我们报告一例患有严重瘙痒的小儿PPV病例，该病例对各种长期口服抗过敏药物和外用他克莫司均无反应，但对血卟啉单甲醚介导的光动力疗法（HMME-PDT）有暂时反应。HMME-PDT是遵循对病变血管壁进行靶向光动力破坏的基本原理用于治疗PWS的最新技术。此外，许多研究已证实HMME-PDT对成人和儿童PWS均具有安全性和有效性。必须进一步探索具体的病理生理机制和治疗方法，以增进我们对该疾病的了解并改善PPV患者的生活质量。
