Aplasia cutis congenita type VI (Barts syndrome), is a rare genetic disorder characterized by congenital localized absence of skin, formation of blisters (epidermolysisbullosa), and nail abnormalities. In this report, we present a rare case of aplasia cutis congenita type VI (Barts syndrome) in a newborn malebaby with the absence of a skin layer over the anterior right leg, slightly below the patella (kneecap) and around the ankle joint.On the second day, the affected areas developed blisters characterizing epidermolysisbullosa.Laboratory investigationswere all normal. The patients wound was managed conservatively with dressing and topical antibiotic ointments.
Aplasia cutis congenita (ACC) is a localized congenital absence of skin with a reported incidence of 3 per 10000 live births. Most common location is the scalp. The diagnosis is made clinically. The management of the lesion depends on the size and most of them are managed conservatively but larger lesions need surgical closure. Although aplasia cutis congenita is rare, it is very important for the general pediatrician to recognize this and consider it in the differential diagnosis of skin lesions specially the lesions on the scalp. We present a newborn infant with scalp lesion which was clinically diagnosed as aplasia cutis congenita and was managed conservatively.