Frieden's Group-V Aplasia Cutis Congenita with in utero Scarring in a Dichorionic Twin: A Case Report and Comprehensive Literature Review.

ABSTRACT:

Introduction

Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by the localized absence of skin at birth, primarily affecting the scalp but also affecting the trunk and limbs. Nine different presentations have been reported. Group V-ACC (G-V ACC) is a rare type associated with fetus papyraceus. The pathogenesis is speculated to involve ischemia from acute hypovolemia or disseminated intravascular coagulation due to thrombotic tissue affecting the surviving twin. Management of ACC depends on lesion severity, focusing on preventing infection and promoting healing, with conservative measures in most cases and surgical intervention in some cases. The long-term prognosis for isolated G-V ACC is generally favorable unless associated with systemic complications.

SUBMITTER: Paraparambil Vellamgot A 

PROVIDER: S-EPMC11658788 | biostudies-literature | 2025 Jan-Dec

REPOSITORIES: biostudies-literature