Abstract
Aplasia cutis congenita was first described by Campbell in 1826. Autosomal dominant as well as autosomal recessive forms of the condition have been reported (2).
The gene locus is unknown. The disorder is visible at birth with the presence of a
sharply defined skin defect most frequently involving the midline scalp. Other sites
including the trunk and extremities have also been observed. The defect in the skin
is often oval or circular, measuring 0.5-5.0 cm, with a glistening red base. The epidermis as well as the dermis may be missing, and ulceration may extend to the deep
subcutaneous tissue (9). Defects vary from a denuded ulcer with a red weeping or
granulating base to an erosion covered with a thin friable membrane. In 70% of
cases, there is a single ulceration. Multiple ulcers are less common. Histologic evaluation reveals the absence of epidermis, paucity of appendageal structures, and a
variable decrease in elastic tissues. In deeper defects, there is a complete deficit of
all layers of skin and subcutaneous tissues.