Multidisciplinary management of a previously unreported presentation of severe aplasia cutis congenita

Aplasia cutis congenita (ACC) is characterized by the complete or partial absence of skin at birth, with 85% of cases of ACC involving the scalp vertex. The etiology of ACC is unclear and appears to be multifactorial. We present the case of a 3-month-old boy who presented with a diagnosis of non-scalp ACC affecting approximately 80% of his total body surface area at birth. This case adds to the literature due to the patient's survival beyond the first day of life and his unique and severe distribution of defects, which led to respiratory compromise and required multidisciplinary management.