Abstract
Aplasia cutis congenita (ACC) is a rare developmental malformation characterized by the absence of skin, and often extends to bone or dura in a localized or widespread area at birth. ACC is seen most commonly on the scalp reported up to eighty-four percent of all cases in the literature. It often manifested as a solitary lesion without other anomalies, but sometimes represented as part of a heterogenous group of disorders. We presented a case of ACC associated with cutis marmorata telangiectatica congenita, secundum atrial septal defect and epilepsy which seem not fit well with any previously recognized clinical syndrome. In addition, emphasis on pathogenesis, histopathology, and treatment strategies were also discussed.
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