A rare case of extensive aplasia cutis congenita: Our surgical approach.

Abstract

Aplasia cutis congenita (ACC) is a rare disorder resulting in the absence of skin or deeper layers, most often involving an isolated small area on the scalp. However, extensive cutis aplasia involving multiple large critical areas of the body is extremely uncommon and remains a challenging condition to manage. Initial concerns involve early mortality from excessive moisture loss, hypothermia, bleeding, sepsis, and brain herniation while subsequent sequelae from delayed wound healing resulting in scarring and loss of function also provide numerous management dilemmas. Conservative treatment with dressings, which typically allows epithelisation in small cases, is inadequate. Surgical approaches described such as skin grafts and rotational flaps are also insufficient in extensive ACC involving the chest and entire scalp. In this article, we present how our centre successfully treated a patient with a large total body surface area of ACC involving the entire scalp, neck, forehead, chest, trunk, lateral flanks, and patchy areas of all four limbs.