Abstract
A 2-year-old girl with fever and tachypnea was noted to have honeycomb lungs on chest X-ray, which was histologically confirmed to be Langerhans cell histiocytosis (LCH) by lung biopsy. The patient also showed skin lesions (histologically positive) and hepatosplenomegaly (biopsy not done); however, no bone lesions were found. In spite of chemotherapy, including prednisolone and etoposide, the lung disease persisted, developing tension pneumothorax, and the patient died of pulmonary failure 6 weeks later. LCH with bilaterally disseminat-ed lung involvement was thought to be difficult to treat. It was not known when the disease started to develop because of no previous chest X-ray; the effects of an early diagnosis and more intensive therapy in such cases remains to be determined.
