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目的:分析儿童穿通型毛母质瘤的临床及病理特点，探讨其发生机制。方法:回顾2014—2020年就诊于北京儿童医院皮肤科的29例穿通型毛母质瘤患儿资料，分析其临床及组织病理学特点。结果:29例中男11例，女18例，男女比例为1∶1.64。发病年龄3个月至14岁10个月，中位发病年龄4.58岁。病程2个月至2年，平均8.72个月。发生穿通时间2 d到1年6个月，平均1.85个月。除1例外用鱼石脂治疗后破溃，3例局部搔抓或磕碰后破损，其余25例均无明确诱因自发破溃。自发病到肿物出现穿通的平均时间为6.87个月。发病部位：面部15例，侧颈部8例，上肢4例，头皮2例。临床上均表现为伴有皮肤结痂或溃疡的皮下质硬结节，其中溃疡型19例，皮角型8例，结痂型2例。肿物直径0.3~2.5 cm，平均1.2 cm。组织病理：肿瘤位于真皮浅中层，主要由嗜碱性细胞及影细胞组成，其中19例表现为瘤体通过穿孔的表皮通道排出皮肤，穿通部位两侧表皮增生并向下凹陷延伸，形成上皮性隧道包绕肿瘤；4例表现为瘤体表面皮肤受压变薄及破溃；6例因手术表皮与瘤体分离，无法观察到穿通部位表现。皮损均予手术切除，术后随访无感染及复发。结论:儿童穿通型毛母质瘤最易发生于面颈部，一般进展快，可分为溃疡型、皮角型及结痂型，组织学提示经表皮消除现象是穿通型毛母质瘤发生穿通的重要机制。“,”Objective:To analyze clinical and pathological features of childhood perforating pilomatricoma, and to explore its pathogenesis.Methods:A retrospective analysis was conducted on clinical and histopathological data from 29 children with perforating pilomatricoma in Department of Dermatology, Beijing Children′s Hospital from 2014 to 2020.Results:Among the 29 patients, 11 were males, and 18 were females, with a male-to-female ratio of 1∶1.64. Their age at onset ranged from 3 months to 14 years and 10 months, and the median age at onset was 4.58 years. The disease duration ranged from 2 months to 2 years, with an average of 8.72 months. The perforation occurred 2 days to 1 year and 6 months after the onset of pilomatricoma, with an average of 1.85 months. Ulceration occurred in 1 patient after the treatment with ichthammol, as well as in 3 patients after local scratching or bumping, and spontaneous ulceration without definite precipitating factors occurred in the remaining 25 patients. The average duration from the onset of disease to tumor perforation was 6.87 months. Skin lesions occurred on the face in 15 cases, on the lateral neck in 8, on the upper limb in 4, as well as on the scalp in 2. Perforating pilomatricoma clinically manifested as indurated subcutaneous nodules with crusts or ulcers, and was classified into 3 subtypes: ulcerative type (19 cases) , horny type (8 cases) , and crusted type (2 cases) . The tumor diameter ranged from 0.3 to 2.5 cm, with an average of 1.2 cm. Histopathological examination showed that the tumor was located in the superficial to middle dermis, and mainly consisted of basophils and ghost cells; in 19 cases, the tumor mass was extruded onto the skin surface through a perforated epidermal channel, and the epidermis around the perforation site was hyperplastic and invaginated into the dermis, forming epithelial tunnels surrounding the tumor; in 4 cases, the skin on the tumor surface was thinned and ruptured; in 6 cases, the perforation site could not be observed due to surgical separation of the epidermis and tumor. All lesions were resected, and no infection or recurrence was observed during the postoperative follow-up.Conclusions:Childhood perforating pilomatricoma mostly occurs on the face and neck, usually with rapid progress, and can be classified into ulcerative type, horny type and crusted type. Histological findings suggest that transepithelial elimination is an important mechanism underlying the occurrence of perforation in pilomatricoma.