Aplasia cutis congenita (ACC) is a relatively rare congenital anomaly that most commonly occurs as a solitary cutaneous defect on the scalp. Depth of involvement varies, and involvement of deeper calvarium and dural structures can be seen in more severe cases. Multiple classification systems have been devised with the Frieden Classification System being the most widely adopted. Using this system, we describe a patient that developed Type V ACC with associated fetal papyraceous. The child healed remarkably well with the application of petrolatum impregnated gauze and topical silver sulfadiazine twice daily for approximately 4 weeks. The child was noted to have no significant contractures or complications at 6-months and 1-year follow-up exams. Herein, we review the current literature on type V ACC including a discussion of treatment options.
© 2017 The International Society of Dermatology.