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Slovenska pediatrija 2021; 28: 30-38

https://doi.org/10.38031/slovpediatr-2021-1-05en

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Review article

CONGENITAL SKIN APLASIA

Aida Zečkanović
Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Sandra Cerar
Klinični oddelek za neonatologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Abstract

Congenital skin aplasia (Aplasia cutis congenita - ACC) is part of a heterogeneous group of conditions characterised by the congenital absence of the epidermis, dermis, and in some cases, subcutaneous tissues, muscles, meninges, or bone, usually involving the scalp vertex. It may present as ulceration, membranous change, or an atrophic scar. ACC can occur as an isolated defect or can be associated with other congenital anomalies. In patients with large scalp and skull defects, there is an increased risk of infection and bleeding along with increased mortality and therefore prompt and effective management is advised. Its aetiology and pathogenesis remain unclear. We discuss the different types of ACC (Frieden’s classification system) and associated anomalies along with an approach to diagnosis and management, which is usually conservative. The authors present a case report of a neonate with ACC due to fetus papyraceus.

Key words: aplasia cutis congenita, congenital anomalies, neonate, aetiology, classification, treatment