Aplasia cutis congenita with fetus papyraceus (Frieden classification Type V)-systematic review of literature and reported cases from 1990 to 2025 with a proposed revised diagnostic criteria and management algorithm.

UNLABELLED: This study evaluated the clinical and obstetric patterns of all reported cases of aplasia cutis congenita (ACC) Type V with fetus papyraceus and vanishing twin syndrome from 1990 to 2025 to assist in creating a diagnostic criteria and revising management algorithm. All case reports published on PubMed between January 1990 and August 2025 were extracted using customized search terms related to ACC Type V, fetus papyraceus, and vanishing twin syndrome. Articles that fulfilled Frieden diagnostic criteria or explicitly diagnosed ACC Type V were included for review. We identified 70 reported cases including demographic, obstetric, clinical, management, and outcome data. The mean gestational age at co-twin demise was 14.21 weeks, and fetus papyraceus was present in only 47.1% of cases. We found that first-trimester demise was strongly associated with truncal "H-pattern" lesions, whereas later gestational age demise more frequently involved scalp and extra-truncal lesions. Assisted reproductive technologies accounted for 15.7% of pregnancies, and 10% followed intentional fetal reduction. Conservative management was effective in most patients, with surgery reserved for extensive or complicated lesions. CONCLUSION: ACC Type V remains underrecognized due to variable presentation and frequent absence of fetus papyraceus at delivery. Therefore, it is crucial for the clinical diagnosis to include a history of multifetal gestation with spontaneous or intentional co-twin demise, along with the characteristic cutaneous findings, even without placental infarction or visible fetus papyraceus. Increasing use of assisted reproductive technologies globally may contribute to the rising incidence of this condition, emphasizing the importance of early recognition and individualized multidisciplinary management. WHAT IS KNOWN: • Aplasia Cutis Congenita Type V is a rare congenital disorder associated with fetus papyraceus and vanishing twin syndrome. • It classically presents with symmetric truncal "H-pattern" skin lesions in the surviving twin. WHAT IS NEW: • This review shows that fetus papyraceus is absent in more than half of reported ACC Type V cases. • Assisted reproductive technologies and intentional fetal reduction may represent emerging contributors to ACC Type V incidence.