Extensive Aplasia Cutis Congenita: A Case Report

Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by localized or widespread skin absence. The most common site is the scalp and the main cause is unknown. Here we report a case of extensive ACC with scalp involvement. A female infant with an Apgar score of 9 and weighing 3406 gr was born by cesarean section in Besat Hospital, Sanandaj, Iran. A lesion measuring 5×10 cm with highly prominent vessels was on the forehead and parietal bone without scalp, skull, or bone tissue leading to the brain tissue being covered with a thin membrane. Other clinical examinations were normal. The newborn suffered from a ruptured aneurysm and intracerebral hemorrhage and underwent pharmacologic treatment. She died after 21 days of hospitalization. In order to prevent mortality in infants with skin defects, infection control is a priority. Antibiotics could be administered in cases with extensive lesions to prevent neonatal sepsis and improve scar tissue formation. Moreover, restorative measures should be taken if necessary.

Keywords:

Aplasia , Congenital , Cutis , Lack of scalp