Brackenrich Jared, Saleh Haitham M., Brown Aleksandra
Lewis Gale - Montgomery
Department of Dermatology, Ain Shams University
Aplasia cutis congenita (ACC) is a rare congenital skin defect involving focal or extensive absence of the epidermis, dermis, and, occasionally, subcutaneous tissue. The exact etiology remains unclear, with proposed mechanisms including impaired prenatal skin development. Initially reported by Cordon in 1767, ACC may present as solitary or multiple lesions on any body region, with 70% to 90% localized to the vertex of the scalp. Classification includes 6 subtypes, some associated with congenital dermatologic syndromes. Most lesions heal spontaneously. However, certain locations and clinical features warrant thorough evaluation to detect underlying soft tissue anomalies that may be life-threatening.
先天性皮肤发育不全（ACC）是一种罕见的先天性皮肤缺陷，其特征为表皮、真皮局部或广泛缺失，偶尔皮下组织也会缺失。ACC的确切病因尚不完全清楚；然而，这可能是由于产前皮肤发育受损所致。该病表现为单发或多发损害，可出现在身体的任何部位，不过70%至90%的损害局限于头皮顶部。ACC已被分为六个亚型，其中一些与先天性皮肤病综合征相关。尽管大多数损害可自愈，但某些部位和临床特征应促使进行更全面的检查，以筛查可能危及生命的潜在软组织异常情况。1986年提出了一种ACC的分类方法，至今仍被接受，如下所示。第1组：无多发畸形的头皮ACC。第2组：伴有肢体异常的头皮ACC。第3组：伴有表皮痣和器官样痣的头皮ACC。第4组：覆盖先天性畸形的ACC。第5组：伴有胎儿纸样化或胎盘梗死的ACC。第6组：伴有大疱性表皮松解症的ACC。第7组：局限于四肢且无水疱的ACC。第8组：由特定致畸物引起的ACC。第9组：与畸形综合征相关的ACC。
