儿童穿通型毛母质瘤29例临床病理特点分析

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...

Analysis of clinical and pathological features of 29 cases of perforating pilomatricoma

【Abstract】 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.