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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

Publication at Faculty of Medicine in Pilsen |
2015

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 negativ clinical finding on one side and massive histopathological finding on the other.