Symmetric Truncal Aplasia Cutis Congenita following Multifetal Reduction of a Sextuplet Pregnancy

Symmetric Aplasia Cutis Congenita Associated with Fetus Papyraceus: Report of Two Cases

“…Placental vascular anastomoses are considered pathogenic in fetus papyraceus‐associated ACC, with 95% (18/19) of reported patients resulting from monochorionic twin pregnancies (4). Current evidence suggests that the surviving fetus suffers from acute hypovolemia after cotwin demise, leading to ischemia of the skin and internal organs, most notably in symmetric “watershed” areas of the trunk and extremities (5).…”

“…Current evidence suggests that the surviving fetus suffers from acute hypovolemia after cotwin demise, leading to ischemia of the skin and internal organs, most notably in symmetric “watershed” areas of the trunk and extremities (5). Other than ACC, fetus papyraceus is also associated with gastrointestinal infarctions in the surviving monochorionic twin (bowel atresias) when cotwin death occurs during the late first to early second trimester, whereas later fetal demise is associated with cerebral infarctions in the surviving fetus (4).…”

“…Only 44 cases of ACC associated with fetus papyraceus have been reported in the literature to date (4). While our cases do not demonstrate the classic large stellate or angulated lesions, they follow the pattern where ACC affecting the trunk was more likely to be associated with death of a monochorionic cotwin before 14 weeks gestation, whereas extratruncal fetus papyraceus‐associated ACC was more common after 14 weeks gestation (2,4). Thus, distribution of ACC lesions on the extremities greater than trunk is a useful marker for a later time of fetal demise, whereas small linear, arcuate, and triangular lesions should also be considered in this presentation of ACC.…”

Truncal aplasia cutis congenita grafted with dermal regeneration matrix – A case report

“…Class V is even rarer and only 45 cases are published in the western literature [1]. Lesions associated with Class V ACC are often bilateral and symmetrical, localized at the trunk, buttocks and thighs, in "H" or "stellate" configuration [2][3][4][5]. The pathophysiology of this disorder is poorly understood with at least five different theories on the etiology of Group V ACC.…”

Aplasia Cutis Congenita in a Setting of Fetus Papyraceus Associated with Small Fetal Abdominal Circumference and High Alpha‐Fetoprotein and Amniotic Acetylcholinesterase

“…In the setting of the death of a twin fetus in the late first or early second trimester of pregnancy, the condition is known as ACC associated with fetus papyraceus (Group 5, Frieden classification) , and the lesions often present as large, stellate, truncal defects . Forty‐nine cases have been reported in the literature.…”