Abstract
INTRODUCTION: Aplasia Cutis Congenita (ACC) is a rare anomaly characterized by absence of portion of skin over localized or widespread area.1 ACC is most often a benign isolated defect,2 but it can be associated with other physical anomalies or malformation syndromes. More than 500 cases have been reported since it was first described, due to significant underreporting of this benign disorder, the precise frequency is unknown. Approximate incidence is 1:10,000 births,3 with no racial or sexual predilection. Scalp is the commonest location and is involved in almost 85% of cases rest 15% cases involve the non-scalp areas like forearms, knees, both sides of the trunk and neck in order of frequency.4,5 About 15% to 30% of the scalp involved patients have skull defects and defects of the dura. Involved areas are non-inflammatory and well demarcated and vary in size from 0.5 to 10 cm or larger. At birth lesions may have already healed and appear as an atrophic, membranous, bullous or parchment like scar with associated alopecia or may remain as a superficial lesion or erode deeply and occasionally involve the meninges.
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