ABSTRACT
Aplasia cutis congenita (ACC) denotes congenitally localized or widespread absence of skin (1,2). Multiple classification systems have been proposed (3) for this heterogeneous group of disorders, which is associated with various other anomalies. Frieden (4) developed a nine-category classification system to group distinct clinical subtypes of ACC. The criteria for distinction between the groups takes into account the location and pattern of skin absence, associated malformations, and the mode of inheritance (4). Characteristically, the defect is a midline solitary lesion that most commonly affects the scalp (86%) without associated anomalies. Sometimes, this defect of the integumentary system extends beyond the dermis to involve the underlying bony calvarium and dura, occurring in approximately 35% of the scalp defect cases (5). Although the true incidence is unknown, ACC is a rare condition believed to occur in about one in 3000 live births (6). Since Cordon’s original article in 1767 (2), greater than 500 cases, have been reported. Cordon’s article was the first to describe ACC of the extremities, followed in 1826 by Campbell who described defects involving the scalp (7).
