Open AccessNeumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans
Author(s) -
Jesús Garcı́a,
Mirna Guadalupe Rios Osuna,
Eduardo Altamirano Álvarez
Publication year - 2014
Publication title -
archivos argentinos de pediatría
Language(s) - Spanish
Resource type - Journals
SCImago Journal Rank - 0.236
H-Index - 19
eISSN - 1668-3501
pISSN - 0325-0075
DOI - 10.5546/aap.2014.e113
Subject(s) - medicine , langerhans cell histiocytosis , pathology , histiocyte , parenchyma , pneumothorax , lung , histiocytosis , eosinophilic , radiology , disease
Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.