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Bart´s syndrome associated with epidermolysis bullosa junctionalis and with pyloric atresia. An autopsy case report


Authors: Katarína Adamicová 1;  Tomáš Balhárek 1;  Lucia Lúčanová 2;  Oľga Nyitrayová 3;  Želmíra Fetisovová 4
Authors‘ workplace: Ústav patologickej anatómie JLF UK a UN Martin 1;  Neonatologická klinika JLF UK a UN Martin 2;  Cytopathos, spol. s r. o., Bratislava 3;  Dermatovenerologická klinika JLF UK a UN Martin 4
Published in: Čes.-slov. Patol., 50, 2014, No. 4, p. 155-158
Category: Original Article

Overview

Bart‘s syndrome, in literature also known under the name CLAS (Congenital Localised Absence of Skin), first described by Bart in 1966 as congenital localized absence of skin, epidermolysis bullosa congenita and nail abnormalities. The authors present a macroscopic and histological findings of a newborn with Bart‘s syndrome, with epidermolysis bullosa junctionalis and atresia pylori, who died 17 days after birth and 13 days after surgery for pyloric stenosis.

Key words:
Bart´s syndrome atresia pylori epidermolysis bullosa junctionalis CLAS


Sources

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