Histiocitosis X pulmonar: Presentacin de un caso y revisin de la literatura

Histiocytosis X or Langerhans cell histiocytosis is an unfrequent disease, which consists on proliferation of Langerhans cells. The etiology is unknown. Diagnosis is reached by electron microscope study of the biopsy, inwhich the Birbeck intracitoplasmatic granules of the Langerhans cells are found and/or by inmunohistochemistry procedures able to detect S-100 antigen and CD 1 cells. Diagnosis can also be reached with a bronchoalveolar lavage in which CD 1 cells will appear in a score higher than 5%. We present the case of a 16 year-old girl that first appeared with a pulmonar lesion with a honey comb X-ray pattern and unsintomatic mandibular bone affectation. Diagnosis was reached by biopsy study using S-100 antigen detection procedures. We consider this case an important one due to the unfrequency of this particular disease.