Congenital Localised Absence of Skin (Bart ’ S Syndrome ) Rare Case Report

Abstract

It is defined as congenital localised absence of skin associated with epidermolysis bullosa. It may be associated with any type of epidermolysis bullosa. Three cutaneous manifestation are characteristics of Bart syndrome: congenital localised absence of skin (CLAS), mucocutaneous blistering and nail abnormalities. It present as raw beefy areas of denuded skin on trauma prone site; extremity like hands and feet, also perioral area. Diagnosis is obvious clinically but requires ultrastructural microscopy. We describe here a case of newborn baby in our OPD who presented with raw beefy red areas of denuded skin on the hands, feet, neck area with few flaccid bulla near thigh. The patient was treated with antibiotic and steroid (fusidic acid + hydrocortisone) cream and emollients. Thus, Clinical appearance was sufficiently distinct to suggest the diagnosis of Bart's syndrome.