Bart Syndrome

which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. A B C Fig. 1. Pretreatment photograph. (A) Extensive absence of skin on the left lower extremity, (B) a ruptured blister on the right hand, and (C) nail deformity of the left great toe. Bart syndrome is a rare inherited disorder characterized by the localized absence of the skin, blister formation, and nail deformity and is considered a variant of aplasia cutis congenita (ACC) with epidermolysis bullosa (EB) [1]. Bart et al. [1] introduced this syndrome in a 1966 report describing a single kinship with a combination of these traits. To the best of our knowledge there are very few cases of Bart syndrome reported in the PubMed database. Here, we present a newborn female baby with Bart syndrome involving ACC in the lower extremities and right hand as well as EB in the right hand and right lower extremity. A female infant was born during the 40th week of the mother's third pregnancy. The patient was referred to the newborn intensive care unit of our hospital, which consulted with the department of plastic surgery because of a skin problem. On physical examination, the patient had extensive absence of skin on the right hand and both lower extremities, a single bullous lesion on the right hand, and three bullous lesions on the right lower extremity. The skin lesion was enveloped in an ultrathin translucent membrane, and there were some blisters on the membrane. Moreover, the patient had a nail deformity of great toe (Fig. 1). The patient's mother had an absence of skin at birth as evidenced by a thin but broad scar on her left shin. Except for the mother's history, there was no other specific family history. A biopsy of the bullous lesion on the edge of the right foot was performed. Light microscopic examination revealed a subepidermal blister, and electron microscopy showed evidence of separation of the dermal-epidermal junction and interruption of the basal lamina, similar to what is seen in junctional EB (Fig. 2). The patient was managed conservatively without operation. When the patient was referred to our hospital, there were crusts and dead tissues on wounds without infection sign. We cleaned crusts and dead tissues using mechanical debridement and hydrodebridement with normal saline and applied antibacterial ointment to protect from infection and to induce wound contraction. Petrolatum …