Document Detail

Systemic aplasia cutis congenita: A case report and review of the literature.
MedLine Citation:
PMID:  20188489     Owner:  NLM     Status:  MEDLINE    
Aplasia cutis congenita (ACC) belongs to a heterogeneous group of conditions characterized by a congenital absence of skin, usually on the vertex of the scalp. It can occur as an isolated defect or can be associated with a number of other congenital anomalies. Two cases of systemic ACC of a more severe and extensive type were recently reported. Here, we describe the third case of systemic ACC and review the available literature. A female infant was born with an extensive defect of the skin, a skull defect, imperforate hymen, and some other anomalies. She died soon after birth probably due to asphyxia and dehydration. We also compared the pathologic findings of the current case with those of the other two previously reported cases. This case suggests that systemic ACC might be recognized as a new syndrome. A limitation is that there is only 1 case, and it is difficult to gain a deeper understanding of its etiology and diagnostic criteria.
Juan Zhou; Li Zheng; Wenqi Tao
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2010-02-26
Journal Detail:
Title:  Pathology, research and practice     Volume:  206     ISSN:  1618-0631     ISO Abbreviation:  Pathol. Res. Pract.     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-07-15     Completed Date:  2010-11-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7806109     Medline TA:  Pathol Res Pract     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  504-7     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Elsevier GmbH. All rights reserved.
Department of Pathology, Shanghai 6th People's Hospital, Shanghai Jiaotong University, 600 Yishan Road, Shanghai 200233, PR China.
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MeSH Terms
Abnormalities, Multiple / pathology*
Ectodermal Dysplasia / pathology*
Infant, Newborn
Scalp / abnormalities
Skin / pathology
Skull / abnormalities

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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