Aplasia cutis congenita (ACC), which is defined as a localized absence of skin at birth is a rare congenital anomaly of the skin affecting one infant in every 10000 live births. To date, just over 500 cases have been reported. In some cases, ACC occurs as a result of placental infarcts or the death of a twin fetus in utero. Sometimes, lesions can be a sign of embryologic malformation, chromosomal abnormality, or ectodermal dysplasia. Association with epidermolysis bullosa, intrauterine infections, or specific teratogens has also been described. Defects of the skin limited to the limbs are rare.
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