title: [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].
abstract: 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.
authors.forename: Daniel
authors.initials: D
authors.lastname: Farkaš
authors.forename: Marián
authors.initials: M
authors.lastname: Švajdler Ml
authors.forename: Lucia
authors.initials: L
authors.lastname: Fröhlichová
authors.forename: Jana
authors.initials: J
authors.lastname: Šprláková
authors.forename: Silvia Farkašová
authors.initials: SF
authors.lastname: Iannaccone
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Szép
authors.forename: Oľga
authors.initials: O
authors.lastname: Nyitrayová
mesh_terms: D000653:Amniotic Fluid; D004820:Epidermolysis Bullosa; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases; D008168:Lung / Q000166:cytology / Q000473:pathology; D016676:Macrophages, Alveolar; D053120:Respiratory Aspiration; D018805:Sepsis; D012867:Skin / Q000473:pathology
keywords: epidermolysis bullosa - foamy alveolar macrophages - amniotic fluid aspiration.