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Unusual lung finding of massive alveolar filling with foamy macrophages in congenital epidermolysis bullosa after amnion fluid aspiration in 15-day-old newborn without any clinical signs of respiratory impairment


Authors: Daniel Farkaš 1;  Marián Švajdler ml. 2;  Lucia Fröhlichová 2;  Jana Šprláková 3;  Silvia Farkašová Iannaccone 4;  Zoltán Szép 5;  Oľga Nyitrayová 5
Authors‘ workplace: Bioptická laboratoř s. r. o., Plzeň, Česká Republika a Oddelenie patológie, Univerzitná nemocnica Louisa Pasteura, Košice, Slovenská Republika ;  Úrad pre dohľad nad zdravotnou starostlivosťou, SLaPA pracovisko, Košice 1;  Šiklův ústav patologie, Univerzita Karlova Praha, Lékařská fakulta Plzeň, Česká Republika 2;  Detská fakultná nemocnica, Košice 3;  Ústav súdneho lekárstva UPJŠ LF, Košice 4;  Cytopathos s. r. o., Bratislava 5
Published in: Čes.-slov. Patol., 51, 2015, No. 2, p. 89-93
Category: Original Article

Overview

Epidermolysis bullosa represents a group of mechanobullous diseases which are most commonly genetically determined. We describe the case of a 15-day-old female newborn with congenital epidermolysis bullosa which was inflicted on aproximately 1/3 of her skin surface, who died because of incorrigible sepsis with multiorgan failure. The main topic of our report is a description of an unusual pulmonary finding of massive alveolar filling with foamy macrophages after amnion fluid aspiration, which contained a excessive amount of desquamated epidermal cells. Introduced case shows outstanding discrepancy of negative clinical finding on one side and massive histopathological finding on the other.

Keywords:
epidermolysis bullosa – foamy alveolar macrophages – amniotic fluid aspiration


Sources

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