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|LETTERS TO EDITOR
|Year : 2018 | Volume
| Issue : 2 | Page : 542-543
Aplasia cutis congenita: A report
H Ibrahim Sun1, F Kaan Aras1, Can Anarat2, Mustafa Güdük1
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 Publication||15-Mar-2018|
Dr. H Ibrahim Sun
Department of Neurosurgery, School of Medicine, Acibadem University, Inonu cad., Okur sok. No: 20, Kozyatagi, Istanbul
Source of Support: None, Conflict of Interest: None
|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
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. 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. 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. 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.
Epithelial ectoderm is the most lacking tissue in ACC. Lesions larger than 3 × 5 cm are considered to be large. Death in cases of ACC is mostly due to superior sagittal sinus hemorrhage.
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 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
Conflicts of interest
There are no conflicts of interest.
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Frieden IJ. Aplasia cutis congenita: A clinical review and proposal for classification. J Am Acad Dermatol 1986;14:646-60.
Spraker MK, Garcia-Gonzalez E, Sanchez LT. Aplasia cutis congenita. In: Schachner LA, Hansen RC, editors. Pediatr Dermatol, 2nd
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[Figure 1], [Figure 2]