Bart's syndrome: a case report

Bart's syndrome is defined as congenital localized absence of skin, oral mucosal lesions and dystrophic nails associated with epidermolysis bullosa. It is an inherited condition as autosomal dominant with complete penetrance but variable expression. We report a rare case of a near term male newborn born to non-consanguineous parents who presented with congenital absence of skin in scalp, face, trunk and extremities. He has dystrophic and deformed fingernails with multiple areas of blistering and erosions. Clinical appearance and skin biopsy was sufficiently distinct to suggest the diagnosis of Bart syndrome