Homa Babaei; Pourang Mohammadi; khadijeh Ramezani-Ali Akbari; Soudabeh Ramezani-Ali Akbari; Zohre Fatemi
Abstract
Background: Aplasia cutis congenital type VI (Bart’s syndrome) has been known as an extremely rare genetic disorder in which there is localized absence of skin, epidermolysis ...
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Background: Aplasia cutis congenital type VI (Bart’s syndrome) has been known as an extremely rare genetic disorder in which there is localized absence of skin, epidermolysis bullosa and nail deformities.
Case report: Here, we present a rare case of Bart’s syndrome in a female newborn diagnosed with congenital loss of skin over upper and lower limbs, trunk, neck and face as well as some bullae on them. Moreover, a dilated stomach was observed in radiographic examination. We treated the baby with TPN, systemic antibiotics, and also her wounds were covered by topical ointments. Laboratory tests, along with liver and renal function analyses were normal, and also serologic tests for infection were negative but she died at the age of 4 days.
Conclusion: The association between Bart’s syndrome and pyloric atresia is a highly fatal combination and there is no treatment option to rescue the patients.
