June 1993

Picture of the Month

Author Affiliations

From the Departments of Pediatrics and Dermatology, The Johns Hopkins University School of Medicine, Baltimore, Md.

Am J Dis Child. 1993;147(6):689-690. doi:10.1001/archpedi.1993.02160300095033

Denouement and Discussion 

Aplasia Cutis Congenita 

Definition  Aplasia cutis congenita is a heterogeneous group of disorders characterized by localized or widespread areas of absent skin at birth. More than 500 cases of this disorder have been reported since the first description in 1767.1

Manifestations  The lesions of aplasia cutis congenita vary in appearance depending on when they occur during intrauterine development, the depth of involvement, and the size and location of the lesions. Those occurring early in gestation may heal before delivery and appear as areas of atrophy, fibrosis, and alopecia. Defects that develop close to delivery usually appear ulcerated or covered by a thin, glistening membrane at birth. Superficial lesions are associated with minimal scarring and hair loss.The size of the lesions can vary considerably from 0.5 cm2 to 100 cm2 2,3 The lesions may occur on any body surface, although most defects appear on the head and 70% to 75% occur as a solitary lesion.4

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