Chen Yan Jing, Yi Qin, Li Yi Ming, Li Li
Department of Dermatology and Venereology, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China.
Clin Cosmet Investig Dermatol. 2021 Dec 16;14:1849-1855. doi: 10.2147/CCID.S338452. eCollection 2021.
Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant-inherited disease characterized by multiple basal cell carcinomas, multiple keratocystic odontogenic tumors, palmar and/or plantar pits. A 50-year-old male patient presented to our hospital with multiple plaques and maculopapular lesions on his face and trunk for more than 20 years. A skin biopsy revealed a number of discrete nests of basaloid cells in the dermis where the peripheral cells are arrayed like a palisade. Multiple odontogenic keratocysts and falx cerebri calcification were found. The diagnosis of NBCCS was made. We treated this patient with 5-aminolevulinic acid photodynamic therapy (ALA-PDT) with red light activation, 5% imiquimod cream and surgical excision for the basal cell carcinomas. All the skin lesions on his face improved substantially after eight sessions of red-light ALA-PDT from clinical observation. Red-light ALA-PDT proved to be a good therapeutic method for NBCCS in this case.
痣样基底细胞癌综合征（NBCCS）是一种罕见的常染色体显性遗传病，其特征为多发性基底细胞癌、多发性牙源性角化囊性瘤、手掌和/或足底凹陷。一名50岁男性患者因面部和躯干出现多个斑块及斑丘疹病变20多年来我院就诊。皮肤活检显示真皮内有许多离散的基底样细胞巢，其周边细胞呈栅栏状排列。发现多发性牙源性角化囊肿和大脑镰钙化。确诊为NBCCS。我们采用5-氨基酮戊酸光动力疗法（ALA-PDT）结合红光激活、5%咪喹莫特乳膏及手术切除治疗该患者的基底细胞癌。临床观察发现，经过8次红光ALA-PDT治疗后，其面部所有皮肤病变均有明显改善。在该病例中，红光ALA-PDT被证明是治疗NBCCS的一种良好治疗方法。
