Aplasia cutis congenita of the scalp: Histopathologic features and clinicopathologic correlation in a case series.,Journal of Cutaneous Pathology

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Aplasia cutis congenita of the scalp: Histopathologic features and clinicopathologic correlation in a case series.
Journal of Cutaneous Pathology ( IF 1.6 ) Pub Date : 2020-01-19 , DOI: 10.1111/cup.13644
Maximilian Gassenmaier ₁ , Hans Bösmüller ₂ , Gisela Metzler ₁

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BACKGROUND Aplasia cutis congenita (ACC) is a rare and heterogeneous disorder characterized by congenital absence of skin. The scalp is the most commonly affected site and lesions may overlie deeper ectodermal abnormalities. The exact etiology is still unknown, and histopathologic features are poorly defined. METHODS A series of 10 cases from nine patients was analyzed to characterize the clinicopathologic spectrum and age-related changes of ACC of the scalp. Hematoxylin and eosin, S100, Elastica van Gieson, and Weigert elastic stains were performed, and clinical information was retrieved from archived medical files. RESULTS Patient ages ranged from 1 day to 39 years (median 57 months). All cases resembled deep-reaching scars with almost complete loss of all adnexal structures. Isolated residual hair follicles were present in 8/10 and sweat glands and ducts in 2/10 cases. The subcutis was thinned or absent. Elastic fibers were always more fragmented than in normal tissue, and the thickness and density increased over time. There was no gain of adnexal structures with increasing age. CONCLUSIONS ACC represents a congenital scarring alopecia with permanent loss of skin appendages. Histopathologic changes resemble a deep-reaching scar with fragmented elastic fibers and differentiate ACC from all other forms of non-traumatic congenital alopecias.

中文翻译：

头皮角质发育不全：病例系列中的组织病理学特征和临床病理相关性。

背景技术先天性皮肤发育不良（ACC）是一种罕见的异质性疾病，其特征是先天性皮肤缺乏。头皮是最常受累的部位，病变可能覆盖更深的外胚层异常。确切的病因仍未知，组织病理学特征定义不清。方法对9例患者中的10例进行分析，以表征头皮ACC的临床病理特征和年龄相关变化。进行苏木精和曙红，S100，Elastica van Gieson和Weigert弹性染色，并从存档的医疗文件中检索临床信息。结果患者年龄为1天至39岁（中位数为57个月）。所有病例均类似于深层瘢痕，几乎所有附件结构完全丧失。在8/10中有孤立的残留毛囊，在2/10例中有汗腺和导管。皮下组织变薄或不存在。弹性纤维总是比正常组织中更碎，并且厚度和密度随时间增加。随着年龄的增长，附件结构没有增加。结论ACC代表先天性瘢痕性脱发伴皮肤附件永久性丢失。组织病理学变化类似于深部瘢痕，弹性纤维断裂，使ACC与所有其他形式的非创伤性先天性脱发相区别。结论ACC代表先天性瘢痕性脱发伴皮肤附件永久性丢失。组织病理学变化类似于深部瘢痕，弹性纤维断裂，使ACC与所有其他形式的非创伤性先天性脱发相区别。结论ACC代表先天性瘢痕性脱发伴皮肤附件永久性丢失。组织病理学变化类似于深部瘢痕，弹性纤维断裂，使ACC与所有其他形式的非创伤性先天性脱发相区别。

更新日期：2020-01-19

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