MEMBRANOUS APLASIA CUTIS WITH HAIR COLLARS - CONGENITAL ABSENCE OF SKIN OR NEUROECTODERMAL DEFECT
MEMBRANOUS APLASIA CUTIS WITH HAIR COLLARS - CONGENITAL ABSENCE OF SKIN OR NEUROECTODERMAL DEFECT
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DOI：
10.1001/archderm.131.12.1427
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发表时间：
1995-12-01
影响因子：
--
通讯作者：
ESTERLY, NB
中科院分区：
文献类型：
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作者：
DROLET, B;PRENDIVILLE, J;ESTERLY, NB
背景：皮肤和神经系统都起源于外胚层。神经外胚层与上皮外胚层的分离与神经管的闭合同时发生。这种时间关系可以解释神经管缺陷上的皮肤异常。一圈深色长发环绕先天性头皮病变（毛领征）就是这样一个标志，往往与脑膨出，脑膜膨出，和异位脑tissue.Observations：Wt描述6名儿童皮肤发育不全谁显示毛领征。皮肤发育不全是一种相对异质性的疾病，然而，这些病变有一个独特的和惊人的相似外观。这种亚型的皮肤发育不全，我们称之为膜性皮肤发育不全，共享几个临床和组织学特征与颅神经管defects.Conclusions：我们建议，膜性皮肤发育不全是一种形式fruste的神经管缺陷，并可能来自类似的胚胎缺陷。对颅神经管闭合的理解的最新进展可能为这一假设提供支持。
Background: The skin and the nervous system are both derived from ectoderm. Separation of neural ectoderm from epithelial ectoderm occurs concurrently with the closure of the neural tube. This chronologic association may explain the cutaneous abnormalities often found overlying neural tube defects. A ring of dark long hair encircling a congenital scalp lesion (the hair collar sign) is one such marker and is often associated with encephaloceles, meningoceles, and heterotopic brain tissue.Observations: Wt describe six children with aplasia cutis who displayed the hair collar sign. Aplasia cutis is a relatively heterogeneous disorder; however, these lesions had a unique and strikingly similar appearance. This subtype of aplasia cutis, which we have termed membranous aplasia cutis, shares several clinical and histologic features with cranial neural tube defects.Conclusions: We propose that membranous aplasia cutis is a form fruste of a neural tube defect and may be derived from a similar embryological defect. Recent advances in the understanding of cranial neural tube closure may provide support for this hypothesis.