Aplasia Cutis Congenita With Epidermolysis bullosa: Bart's Syndrome-Following Line of Blaschko's-A Rarely Reported Disorder

Abstract

Bart's syndrome, first described by Bart, consists of aplasia cutis congenital (ACC), congenital epidermolysis bullosa (EB) and associated nail abnormalities. ACC with EB is a relatively rare condition requiring early diagnosis and treatment. A full term new born female, presented with total loss of skin in S-shaped pattern on both the legs, along the lines of blaschko's. On the second day, few bulla and blisters developed on chest, gluteal region and extremities. ACC was diagnosed clinically and skin biopsy from fresh blister showed features suggestive of EB. Daily wound dressing with systemic antibiotics showed re-epithelialization of limb defect. Bart's syndrome presenting along lines of blaschko's is a rare presentation of a rare disorder.