Lungs „Hassalloid´s-like“ bodies in children with epidermolysis bullosa junctionalis and bart´s syndrome

Overview

Epidermolysis bullosa and Bart´s syndrome are fairly accurately documented diseases by histopathology. In the article the authors describe interesting and hitherto undescribed phenomenon in the lungs male infant with epidermolysis bullosa junctionalis and Bart‘s syndrome, who died 17 days after birth and 13 days after surgery for pyloric atresia, on multiorgan failure within basic congenital diseases.

Histologically in lung alveoli was found to the massive presence of foamy macrophages and numerous globoid formations resembling morphological and immunohistochemical „Hassall´s” bodies in a thymus of the newborn. It was a acidophillic spherical bodies concentric tracks in the connective tissue with focal presence of fibrin, as a unique proof CKAE1/AE3 and CKHMW positive epithelial cells and CD68-positive histiocytic elements. An interesting finding was the follicular skin structure in the center „hassalloid´s-like” body, which suggests an aspiration components of the skin during intrauterine life.

Normal Apgar score at birth of the child (10/10/10 s.) and severe histological features on the death of the child testify for the first pathogenetic formation „hassalloid´s-like” bodies in the lungs during the 17-day life of a disabled child.

Keywords:
epidermolysis bullosa junctionalis – Bart´s syndrome – „hassalloid´s-like“ bodies in the alveoli of the lungs – foamy macrophages