Bart syndrome: The congenital localized absence of skin may follow the lines of Blaschko. Report of six cases

Three cutaneous manifestations are characteristic of Bart syndrome: congeni tal localized absence of skin (CLAS), mucocutaneous blistering, and nail ab normalities. Six cases of Bart syndrome are herein reported. Localized abse nce of skin is present at birth, particularly on the anterior aspects of th e lower extremities and dorsa of the feet. Physical trauma in utero has bee n proposed as a mechanism to explain the denuded areas on the limbs. The re current, highly similar pattern of the congenital defect in regard to locat ion and clinical appearance in our patients and in most of the reported cas es strongly suggests that trauma is too simplistic an explanation. Because of the observed bilateral and symmetric distribution of denuded areas in an S-shaped broad band, their sharply demarcated borders, the involvement of the toe webs, and the frequent similar involvement of the soles, we suggest that congenital localized absence of skin in Bart syndrome may follow the lines of Blaschko.