Aplasia Cutis Congenita of the Scalp with a Familial Pattern.

Waleed Alshehri,Sara Alfadil,Shabeer A Wani,Sari M Rabah,Abdulaziz O Alabdulkarim,Alhanouf Alothri

doi:10.1155/2016/4264721

Abstract

Aplasia Cutis Congenita (ACC) is a condition characterized by congenital absence of skin, usually on the scalp. ACC can occur as an isolated condition or in the presence of other congenital anomalies. Here we describe a case of a 16-day-old baby girl with an isolated ACC of the scalp. Her elder two siblings have been diagnosed with ACC with concomitant cardiac or limb anomalies. The patient was managed conservatively until the defect has formed scar tissue 6 months later.

Highlights

Cutis aplasia or Aplasia Cutis Congenita (ACC) is an uncommon and rare congenital abnormality involving variant layers of the skin, mostly as a solitary lesion involving the midline over the skull vertex and, less commonly, underlying periosteum and bone [1]
Aplasia Cutis Congenita is a rare congenital disorder characterized by absence of skin, most frequently overlying the scalp
ACC can be associated with many anomalies

Summary

Introduction

Cutis aplasia or Aplasia Cutis Congenita (ACC) is an uncommon and rare congenital abnormality involving variant layers of the skin, mostly as a solitary lesion involving the midline over the skull vertex and, less commonly, underlying periosteum and bone [1]. Frieden classified the different anomalies into 9 groups based on the number and the presence or absence of other anomalies (Table 1) [5] The lesions in those cases are quite variable, ranging from only local absence of skin to a complete absence of epidermis, subcutaneous tissue, bone, or in some cases the dura [6,7,8]. Hairless or scarcely haired scars mandate excision of the lesion and covering it with local flap from the scalp [9, 15,16,17,18,19,20]

Case Report

Discussion

Conclusion