Aplasia Cutis Congenita Associated with Fetus Papyraceus

Abstract

Aplasia cutis congenita (ACC), or congenital absence of skin, is a heterogeneous condition that may be due to varied mechanisms. ACC has rarely been reported in association with fetus papyraceus, representing a peculiar phenotype of ACC. We present six new cases of neonates born with symmetric ACC associated with intrauterine fetal demise of co-twins during the late first or early second trimester.

A Case Report: Aplasia Cutis Congenita Secondary to Fetus Papyraceus

“…Treatment is generally tailored to the extent of the disease. For large, nonscalp lesions like in our case of ACC-FP, the defects can usually be managed conservatively [10,14]. Early diagnosis of the condition by the obstetrician or neonatal provider at the time of birth is imperative for proper management.…”

Extensive Aplasia Cutis Congenita Encircling the Trunk Associated with Fetus Papyraceus

“…Intrauterine trauma is also a possibility although majority of cases do not have a history of intrauterine trauma. More likely, decreased or falling blood pressure of the dying twin leads to exsanguination and acute hypovolemia of the surviving twin with resultant ischemia of the skin and consequent aplasia cutis congenita [ 4 , 10 , 13 , 14 ]. The characteristic involvement of the trunk and extremities is thought to represent watershed areas that are farthest from the vascular supply and therefore most susceptible to ischemic insults in the setting of hypovolemia [ 8 , 15 ].…”

A newborn with a congenital ulcerated plaque on the abdomen

“…However, an association with various congenital extracutaneous abnormalities has been rarely reported 5 . They include bowel atresia, lung anomalies, clubbed hands and feet, nail dystrophy, and various central nervous system abnormalities including cerebral infarction, developmental delay, spastic paralysis, polymicrogyria, subependymal nodular heterotropia, and posterior parietal cortical dysplasia 5 . In the presence of multiple neurological abnormalities, Adams‐Oliver syndrome needs to be ruled out.…”