Abstract
The Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon pulmonary diffuse cystic disease. This review presents one case of PLCH diagnosed in a non-smoker female adolescent, who consulted for rapidly progressive dyspnea associated to left pleuritic pain that corresponded to a bilateral pneumothorax. The diagnosis was confirmed by surgical biopsy of the lung. Treatment with corticoids (prednisone) was indicated during the first year. After 18 years from diagnosis she presented an important clinical improvement, with a favourable but not complete radiological and spirometric improvement. The cases that make their debut with recurrent spontaneous pneumothorax are infrequent. The natural evolution of this disease is variable and the treatment is still controversial, been the immunosuppressive therapy, as corticoesteroids and cytotoxic agents of limited value, since are few studies that confirm their effectiveness
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