Dror Y, Gelman-Kohan Z, Hagai Z, Juster-Reicher A, Cohen R N, Mogilner B
Department of Neonatology, Kaplan Hospital, Rehovot, Israel.
Am J Perinatol. 1994 Mar;11(2):149-52. doi: 10.1055/s-2007-994576.
Aplasia cutis congenita affecting the elbows, knees, hips, and gluteal area was observed in a female newborn, product of a twin pregnancy. One of the twins was a fetus papyraceous detected at 15 weeks of pregnancy. During the course of the pregnancy, maternal thrombocytosis was diagnosed and treated with aspirin. alpha-Fetoprotein was elevated in maternal serum and amniotic fluid, and a distinct electrophoretic acetylcholinesterase band was seen in amniotic fluid. These findings are in agreement with the classification of aplasia cutis congenita as proposed by Frieden et al in which type V is related to the presence of a fetus papyraceous or placental infarcts. The findings in the present case may be explained by the effect of the dead twin on the surviving fetus and the extensive denuded skin areas. Long-term follow-up of the infant showed that the lesions were cured, most of them with minimal scars. Increased risk for aplasia cutis congenita should be considered when elevated maternal and amniotic fluid alpha-fetoprotein and a distinct electrophoretic band of acetylcholinesterase are found. Especially when one of the twins is dead.
在一名双胎妊娠的女新生儿中观察到先天性皮肤发育不全，累及肘部、膝部、髋部和臀区。双胞胎之一是在妊娠15周时检测到的纸样胎儿。在妊娠过程中，诊断出母体血小板增多症并用阿司匹林进行了治疗。母体血清和羊水中甲胎蛋白升高，并且在羊水中可见一条明显的电泳乙酰胆碱酯酶带。这些发现与Frieden等人提出的先天性皮肤发育不全分类一致，其中V型与纸样胎儿或胎盘梗死的存在有关。本病例的发现可能是由于死胎对存活胎儿的影响以及广泛的皮肤裸露区域所致。对该婴儿的长期随访表明，病变已治愈，大多数留下的疤痕极小。当发现母体和羊水甲胎蛋白升高以及一条明显的电泳乙酰胆碱酯酶带时，应考虑先天性皮肤发育不全的风险增加。特别是当双胞胎之一死亡时。
