儿童水疱型毛母质瘤16例临床病理分析

目的 分析儿童水疱型毛母质瘤的临床病理特点。方法 对2013—2017年就诊于北京儿童医院皮肤科的16例水疱型毛母质瘤患者进行临床病理分析。结果 16例患者中，男5例，女11例。发病年龄4个月至11岁，中位发病年龄8.5岁。病程2个月至4年，平均病程10个月。发病部位：上肢10例（上臂7例、肩部2例、前臂1例），面部4例，颈部2例。皮损表现为局限性可推动的红色肿物，呈水疱样外观，部分皮损表面可见毛细血管扩张，肿物直径0.5 ~ 3 cm，触诊水疱内可及质硬结节。皮肤镜下，16例患者皮损见红色均匀背景，13例见白色无结构区，4例见蓝灰色无结构区，11例见线状或树枝状不规则血管，15例患者同时有多种皮肤镜表现。皮损均予手术切除，随访1 ~ 5年无复发。组织病理：肿瘤位于真皮中下部，主要由基底样细胞及影细胞组成，还可见介于上述两种细胞之间的过渡细胞。肿瘤间质中见不同程度炎症细胞浸润及纤维结缔组织增生和钙化，部分可见多核巨细胞。表皮和瘤体之间真皮内见不同程度的炎症细胞浸润、淋巴管扩张及弹性纤维减少或缺失。结论 儿童水疱型毛母质瘤好发于上肢及面颈部，组织病理特点为肿瘤由基底样细胞及影细胞构成，真皮内淋巴管扩张、弹性纤维减少，皮肤镜下以红色背景、白色无结构区为特征。

Clinical and pathological features of 16 cases of childhood bullous pilomatricoma

Sun Juan, Xu Jiaosheng, Wei Li, Han XiaofengDepartment of Dermatology, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing 100045, ChinaCorresponding author: Han Xiaofeng, Email: peak_h@aliyun.com【Abstract】 Objective To analyze the clinical and pathological features of childhood bullous pilomatricoma. Methods The clinical and pathological features were analyzed in 16 patients with bullous pilomatricoma, who visited Department of Dermatology of Beijing Children′s Hospital from 2013 to 2017. Results Among the 16 patients, 5 were males, and 11 were females. Their age of onset ranged from 4 months to 11 years, and the median age of onset was 8.5 years. Their course of disease ranged from 2 months to 4 years, and the average course of disease was 10 months. The tumors were found predominantly on the upper limbs （10 cases, including 7 on the upper arm, 2 on the shoulder and 1 on the forearm）, followed by the face （4 cases） and the neck （2 cases）. These tumors manifested as limited pushable red lumps with blister-like appearance, and telangiectasia was observed on the surface of some lesions. The diameters of the lumps ranged from 0.5 cm to 3 cm, and hard nodules could be detected in the blisters by palpation. Under dermoscopy, uniform red background was observed in 16 cases, unstructured white area in 13, unstructured blue-grey area in 4, and liner or irregular branched vessels. Two or more dermoscopic features were observed in 15 patients. All the skin lesions were resected by surgery, and no recurrence was observed during the follow-up of 1 - 5 years. Histopathological examination showed that the tumors were located in the middle to deep dermis, and mainly consisted of basaloid cells and shadow cells, as well as transitional cells between the above two kinds of cells. Varying degrees of infiltration of inflammatory cells and hyperplasia and calcification of the fibrous connective tissue were observed in the tumor interstitium, with multinucleated giant cells in some areas. There were varying degrees of infiltration of inflammatory cells, lymphangiectasis, reduction or absence of elastic fibers in the dermis between the epidermis and tumors. Conclusions Childhood bullous pilomatricoma mostly occurs on the upper limbs, face and neck. Histopathologically, the tumor consists of basaloid cells and shadow cells with lymphangiectasis and reduction of elastic fibers in the dermis. The main dermoscopic features are red background and unstructured white areas. Its prognosis is good after surgical excision.