皮肤脑脊膜异位8例临床及组织病理分析  

Clinicopathologic analysis of eight cases of cutaneous meningeal heterotopia

作　　者：刘宇 张金娥 刘玲 王雷 王刚 陈凤鸣 LIU Yu;ZHANG Jin'e;LIU Ling;WANG Lei;WANG Gang;CHEN Fengming(Department of Dermatology,Xijing Hospital,Air Force Medical University,Xi'an 710032,China)

机构地区：[1]空军军医大学西京皮肤医院,陕西西安710032

出　　处：《临床皮肤科杂志》2024年第5期262-266,共5页Journal of Clinical Dermatology

摘　　要：目的:探讨皮肤脑脊膜异位(cutaneous meningeal heterotopia,CMH)的临床、组织病理特征及鉴别诊断。方法:对2017年5月至2019年5月在西京皮肤医院确诊的8例CMH患儿的临床及组织病理资料进行回顾性分析。结果:患儿中7例男性,1例女性,就诊年龄9月龄~10岁。7例(87.5%)皮损位于头皮上,另1例(12.5%)位于肩胛间区脊柱正中线上。头皮病变中,有6例为头顶区,1例位于顶额区。4例表现为扁平脱发区(圆形、类圆形及梭形),具有瘢痕样外观;2例表现为扁平脱发区,表面红斑;1例表现为红色光滑结节伴脱发。有5例毛发领圈征阳性。组织病理主要包括4个病变特征:纤维区域8例(100%)、假血管区域8例(100%)、假黏液样区域7例(87.5%)及细胞/合胞体样区域7例(87.5%)。有时可见胶原小体(5例,62.5%)、钙化区域(1例,12.5%)及毛发呈离心性倾斜(5例,62.5%)。可伴有黑素细胞(5例,62.5%)和(或)毛细血管增生(6例,75%)。免疫组化可见上皮膜抗原(epithelial membrane antigen,EMA)、神经元特异性烯醇化酶(neuron-specific enolase,NSE)阳性,D2-40也常见阳性。结论:CMH罕见,常表现为头皮的瘢痕性秃发区,毛发领圈征阳性高度提示该病。当临床怀疑该病时,应在手术前对病变进行影像学检查,以排除颅内扩散或颅骨缺损。Objective:To investigate the clinicopathological features and differential diagnosis of cutaneous meningeal heterotopia.Methods:The clinical and pathological data of eight cases of cutaneous meningeal heterotopia,from Department of Dermatology,Xijing Hospital from May 2017 to May 2019,were retrospectively analyzed.Results:There were 7 males and 1 female diag-nosed at ages 9 months to 10 years.The lesion was located on the scalp in seven cases(87.5%),and the dorsomedian line in one case(12.5%).Among the scalp lesions,six lesions were located in the parietal region and one in the parietal frontal re-gion.Four cases showed flat alopecia area(round,oval,fusiform)with scar-like appearance,two showed flat alopecia area with superficial erythema,and one case showed red smooth nodules with alopecia.Five cases were positive for hair collar sign.The histopathological analysis of onychopapilloma showed fibrous(8 cases,100%),pseudovascular(8 cases,100%),cellular(7 cases,87.5%)and pseudomyxoid(7 cases,87.5%)features.Other features included collagen bodies(5 cases,62.5%),calcifica-tions(1 case,12.5%),dermal melanocytes(5 cases,62.5%)and hyperplasia of capillaries(6 cases,75%).Conclusions:Cuta-neous meningeal heterotopia is rare,often manifested as scarring bald areas of the scalp,and a positive hair collar sign is highly suggestive of the disease.When the disease is clinically suspected,imaging studies should be performed prior to surgery on the lesion to rule out intracranial extension or skull defect.

关 键 词：皮肤脑脊膜异位 临床病理分析 脱发 毛发领圈征 

分 类 号：R758.5[医药卫生—皮肤病学与性病学]