Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 2975  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,118 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this Article
   Article Figures

 Article Access Statistics
    PDF Downloaded30    
    Comments [Add]    

Recommend this journal


Table of Contents    
Year : 2018  |  Volume : 66  |  Issue : 2  |  Page : 542-543

Aplasia cutis congenita: A report

1 Department of Neurosurgery, School of Medicine, Acibadem University, Istanbul, Turkey
2 Department of Psychology, School of Medicine, Acibadem University, Istanbul, Turkey

Date of Web Publication15-Mar-2018

Correspondence Address:
Dr. H Ibrahim Sun
Department of Neurosurgery, School of Medicine, Acibadem University, Inonu cad., Okur sok. No: 20, Kozyatagi, Istanbul
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.227325

Rights and Permissions

How to cite this article:
Sun H I, Aras F K, Anarat C, Güdük M. Aplasia cutis congenita: A report. Neurol India 2018;66:542-3

How to cite this URL:
Sun H I, Aras F K, Anarat C, Güdük M. Aplasia cutis congenita: A report. Neurol India [serial online] 2018 [cited 2021 Jan 15];66:542-3. Available from:


Aplasia cutis congenita (ACC), a congenital absence of all the skin layers, is mostly found on the scalp as a single lesion, but other areas might also be affected.[1] In severe cases, the bone and dura are also absent. Focal ulcerations are the most common presentation of ACC at birth, and diagnosis is most often established by the findings of a smooth, hairless skin defect with eschar or atrophic tissue, by families and medical staff after a few days of birth.[2] Small areas can heal in time with alopecic scars but large areas commonly are prone to infection, trauma, as well as precipitation of seizures and thrombosis; thus, surgical intervention is inevitable.

Nine groups have been proposed by Frieden to distinguish ACC based on clinical features.[3] We present the case of a 2-day old male patient with a 10 × 15 mm lesion on the scalp, belonging to Frieden's group one. The pathophysiology of ACC is not clear yet, although infection, trauma, teratogenic medications, and vascular compromise have been implicated.[4]

Epithelial ectoderm is the most lacking tissue in ACC.[3] Lesions larger than 3 × 5 cm are considered to be large. Death in cases of ACC is mostly due to superior sagittal sinus hemorrhage.[3]

A one-day old male child had a skin defect on the occipitoparietal area that was noted at birth. This skin defect is illustrated in [Figure 1]. The skin defect was at the area of the occipital fontanelle, and hence there was no bone protection under the skin. Ultrasound imaging showed no neurological deficit but the dura was visible directly; surgery was planned on the first day of his life. The inner layer of the defect was incised around the area and matrix suture was used for the closure [Figure 2]. The postoperative course was uneventful. There were no other organ abnormalities. The patient was discharged from the hospital on the first day of the surgery.
Figure 1: Preoperative lesion of the patient (10 × 15 mm)

Click here to view
Figure 2: The inner layer of the defect was incised around the area and matrix suture was used for the closure

Click here to view

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Tempark T, Shwayder T. Aplasia cutis congenital with fetus papyraceus: Report and review of the literature. Int J Dermatol 2012;51:1419-26.  Back to cited text no. 1
AlShehri W, AlFadil S, AlOthri A, Alabdulkarim AO, Wani SA, Rabah SM. Aplasia cutis congenita of the scalp with a familial pattern. Case Rep Surg 2016;2016:4264721.  Back to cited text no. 2
Frieden IJ. Aplasia cutis congenita: A clinical review and proposal for classification. J Am Acad Dermatol 1986;14:646-60.  Back to cited text no. 3
Spraker MK, Garcia-Gonzalez E, Sanchez LT. Aplasia cutis congenita. In: Schachner LA, Hansen RC, editors. Pediatr Dermatol, 2nd edn., vol. 2. Churchill Livingstone: New York, 1995, pp 905-7.  Back to cited text no. 4


  [Figure 1], [Figure 2]


Print this article  Email this article
Online since 20th March '04
Published by Wolters Kluwer - Medknow