APLASIA CUTIS CONGENITA (ACC) AND SEIZURE IN A PREMATURE NEONATE: COULD IT BE A NEW NEUROCUTANEOUS SYNDROME?

One of the few conditions associated with skin ulceration in the neonatal period is aplasia cutis congenita (ACC). ACC or congenital absence of the skin is considered an uncommon anomaly. This malformation commonly appears on the scalp as a solitary lesion, though it can be seen in other surfaces of the body such as the trunk, limbs and face. ACC can be associated with other physical anomalies such as defects of the heart, gastrointestinal system, genitor-urinary system, central nervous system, and also in association with umbilical hernia. There are very few reports of ACC and seizure as a prominent clinical manifestation. In this study, we present a premature neonate with ACC lesions on the right side of the nose, on the right hand and the foot, together with frequent seizures with onset within the initial hours of the birth. Laboratory testing and brain MRI of the patient revealed no significant results. This case may present a new group of ACC classification, or a new neurocutaneous syndrome.