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LETTERS TO EDITOR
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
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.227325

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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 2019 Oct 17];66:542-3. Available from: http://www.neurologyindia.com/text.asp?2018/66/2/542/227325




Sir,

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)

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

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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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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
    
2.
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
    
3.
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
    
4.
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
    


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