Abstract

MARTINEZ GARCIA, Jesús Javier; RIOS OSUNA, Mirna Guadalupe  and  ALTAMIRANO ALVAREZ, Eduardo. Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis. Arch. argent. pediatr. [online]. 2014, vol.112, n.3, pp.e113-e116. ISSN 0325-0075.  http://dx.doi.org/10.5546/aap.2014.e113.

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.

Keywords : Spontaneous pneumothorax; Pulmonary Langerhans cell histiocytosis.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )