Introduction::Pilomatricoma is usually a solitary, deep dermal firm nodule with an overlying normal epidermis. Bullous pilomatricoma is rarely reported; thus, its pathogenesis has not been elucidated.Case presentation::A 19-year-old female presented a firm nodule covered by a bulla on her right upper arm. Histopathological examination showed the tumor was composed of basophilic basal-like cells and shadow cells. No cellular atypia or abnormal mitotic figures were observed. All these clinical characteristics and pathological results point to the diagnosis of bullous pilomatricoma. The tumor was surgically removed by enlargement.Discussion::We hypothesize that a history of trauma and rapid growth may contribute to the diagnosis of the bullous pilomatricoma. The appearance of the bullous can be explained by the dilated lymphatic vessels seen in histopathologic examination. We give a case report help clinicians to better diagnose this entity and decrease the rate of misdiagnosis.Conclusion::We observed a rare bullous variant of pilomatricoma. Hence, when identifying a patient of bullous pilomatricoma, a detailed medical history and physical examination are essential.

目的:探讨局部皮下筋膜蒂V-Y皮瓣在面部皮肤缺损修复中的应用和效果。方法:回顾性分析2012年3月—2021年4月广东省中西医结合医院耳鼻喉科收治并行面部V-Y皮瓣修复的患者23例，其中男性14例，女性9例，年龄45~85岁。病变部位包括面颊部12例（4例涉及下睑），鼻部4例，唇部3例，颞部2例，颏部2例。病理诊断包括基底细胞癌7例，鳞状细胞癌2例，恶性黑色素瘤1例，皮肤角化病7例，皮内痣3例，毛母质瘤1例，混合瘤1例，表皮囊肿1例。根据缺损部位设计合适的皮下筋膜蒂V-Y皮瓣进行修复。总结该皮瓣的血供优势、成活率及不良事件的发生情况；应用χ2检验对不同类型患者的观察结果进行统计学分析。结果:23例均经手术切除原发病灶，术中冷冻病检尽量取得切缘阴性，本组有1例多次切除后仍有1个切缘阳性。缺损面积14 mm×12 mm~59 mm×54 mm。全组皮瓣均成活。本组出现的不良事件是“Y”形三臂会合处及附近表皮少部分坏死结痂共4例，经湿敷、换药后脱痂愈合；其余患者创面一期愈合。不良事件的发生率上根据皮瓣的双蒂与单蒂（4/19比0/4）、良性病变与恶性病变（4/13比0/10）以及患者有基础病与无基础病（1/6比3/17）进行比较差异均无统计学意义（χ2值分别为0.98、3.56、0.01， P值均>0.05）。全组无裂开、血肿、睑外翻、唇部变形等并发症。良性患者随访3个月以上；恶性肿瘤患者随访6~36个月，无复发。 结论:对于面部中小型缺损的修复，如处理恰当，V-Y皮下筋膜蒂皮瓣有可能成为“不易坏死”的局部皮瓣。

Introduction::The most common cutaneous adnexal tumors in children were follicular, especially pilomatricoma, and a few were predominant glandular/ductal differentiation, malignant forms are occasionally encountered.Case presentation::A 10-year-old male child was presented with a nodular in posterior occipital for half a year with no symptoms. Histopathology showed there was acanthosis in epidermis, partially with local ulceration and crusting; and in the dermis, there was irregular scattered or agglomerated infiltration of diffused epithelial cells, partly presenting as basaloid, but no obvious peripheral palisading arrangement; and in the center there was extensive necrosis; cellular pleomorphism, scattered mitotic figures, focal clear cell areas, and adenoid differentiation can also be seen, there was scattered infiltration of mixed inflammatory cells in the stroma. Immunohistochemistry showed cytokeratin (CK) 5/6 +, CK 8/18 +, epithelial membrane antigen +, gata3 transcription factor 3 +, cell adhesion15 (focal +), Ki67 ( +, 30%), carcinoembryonic antigen (focal +), CK 7 (focal +), gross cystic disease fluid protein-15 -, P63 +, S-100 -. Final diagnosis was the malignant cutaneous adnexal tumor with eccrine differentiation, most likely the nodular clear cell hidradenocarcinoma. The patient has no special discomfort follow-up observation after extended resection and lymph node examination. Discussion::The histopathology showed infiltrative growth pattern, deep extension, necrosis, nuclear pleomorphism, mitoses, desmoplastic stromal reaction and the clear cell area and adenoid differentiation. Immunohistochemistry was positive for CK8/18, EMA, CK5/6, P63, gata3 transcription factor 3 and negative for S-100 and GCDFP-15, some gland-derived markers such as CK7, CEA were focal positive, and we have not found the preexisting benign poroma and porocarcinoma in situ, so we preferred the diagnosis of hidradenocarcinoma. The differential diagnosis such as porocarcinoma, clear cell squamous cell carcinoma, and basal cell carcinoma were taken into account.Conclusion::The diagnosis was challenging by clinical manifestations. Histopathology and immunohistochemistry should be combined with clinical presentation, history to reach the final diagnose.

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。

患者女,17岁,右侧肩部水疱样肿物2个月.皮肤科情况:右侧肩部可见一约2 cm ×4 cm大小囊性肿物,中央可触及一质硬结节,边界清.皮肤组织病理示:真皮中可见肿瘤团块,由嗜碱性细胞、影细胞及钙化灶构成,可见淋巴管增生、扩张的淋巴管.诊断:水疱型毛母质瘤.予以手术切除,随访2年无复发.

汇报5例水疱型毛母质瘤(BPM)病例,并分析其皮肤镜特征以及与其他临床易误诊疾病皮肤镜鉴别.BPM皮肤镜下特征主要表现为同时存在的淡色-红色背景,亮黄色不规则团块样结构,团块周边可见无结构的蓝灰色区域;另外可见散在线状不规则血管和亮白色条纹.BPM皮肤镜下特征有利于与其他易混淆疾病鉴别.

目的 探讨毛母质瘤的超声征象与分型及病理表现的相关性.资料与方法 回顾性分析 2014 年 7 月-2021 年 8 月河南省中医院及河南中医药大学第一附属医院共 76 例(78 个病灶)术后经病理证实的毛母质瘤的临床、超声及病理表现,根据声像图有无钙化及钙化模式,分为无钙化型、点状钙化型、片状钙化型及完全钙化型.结果 毛母质瘤以10岁以下儿童最为多见,发病部位多位于头面部.多数病灶周边伴有低回声晕及钙化,78 个病灶中 51 个病灶有不同程度钙化,占 65.4%;36 个病灶钙化伴有低回声晕,63个病灶内部以低回声为主,占80.8%,不同分型毛母质瘤的低回声晕(χ2=15.624,P=0.001)和内部回声(χ2=12.801,P=0.021)差异有统计学意义;78个病灶中均检出影子细胞和嗜碱性细胞,54个超声显示低回声晕的病灶中50个病灶周边发现嗜碱性细胞,不同分型的毛母质瘤超声表现与病理构成有相对应的特征性改变.结论 各型毛母质瘤的超声表现有所不同,与病理改变有相关性.掌握不同分型毛母质瘤的声像图表现有助于术前诊断.

钙化上皮瘤(calcified epithelioma,CE)也称为毛母质瘤(pilomatricoma,PM),是起源于毛囊外根鞘细胞的外胚层良性肿瘤.一般单发于上肢、躯干及面颈部,多发性钙化上皮瘤在临床上更是罕见.本文报告 1 例多发性钙化上皮瘤并进行相关基因检测,结合相关文献进行讨论,为临床诊疗提供参考.

患者女,43岁.项部肿物10年,偶有豆腐渣样物质流出,渐增大.皮肤科情况:项部可见一约2.5cm×1.Ocm的界限清楚的暗红色肿物,局部上覆痂皮,表面散在白色内容物,质硬.经组织病理检查诊断为穿通性毛母质瘤.

目的 探讨毛母质瘤的临床表现及超声特点.方法 回顾性分析我院3年间经手术病理证实的48例,共53个毛母质瘤的临床表现及超声特点.结果 48例53个结节中,术前及术中临床诊断符合率0％,超声诊断符合率18.9％ (10/53);超声检查53个结节内部或边缘伴有钙化的占77.4％ (41/53),周围有低回声晕占77.4％ (41/53),35个结节内部及边缘见条状血流信号,其中11个结节血流丰富.超声对伴有钙化的毛母质瘤诊断率明显提高(80％～100％),对无钙化的毛母质瘤诊断率仍很低(0％～20％).结论 毛母质瘤临床表现多样,容易误诊.超声声像图具有特征性,熟悉其超声表现可以提高术前诊断率.