APLASIA CUTIS CONGENITA TYPE I - A CASE SERIES

Aplasia Cutis Congenita is a rare disorder with circumscribed, partial or widespread absence of skin and subcutaneous soft tissue; in about 20% it also causes skull defects. The disease is heterogeneous in its clinical presentation with nine major subtypes. Type I represents nonsyndromic Aplasia Cutis Congenita. We report 5 infants with skin defects of the scalp and limbs presented to dermatologists. Pediatric workup ruled out any other malformations or genetic disorders. All patients were treated by conservative wound and skin care without complications. In one case the formation of milia has been observed - an outcome not described before. Therapeutic approach and differential diagnoses are described. Topical wound and skin care resulted in complete closure of the defects. Skin appendages did not recover, leaving hairless areas on the scalp and limbs. Aplasia Cutis Congenita type I is a rare disorder in newborns with >85% of all solitary lesions occurring on the scalp. Conservative treatment is a simple and safe option in many cases. Exposed large veins and sagittal plexus demand urgent surgical approaches to prevent fatal hemorrhages or infections.