Unifocal Langerhans Cell Histiocytosis Presenting as Spontaneous Pneumothorax

Langerhans cell is a rare disease affecting the lungs which has a 10 years survival chance having a prognosis of 85%. It is often linked with smoking. We discuss here the correct stepwise approach towards its diagnosis

Presenting a 28-year-old male smoker with a history of shortness of breath for 2 months which worsened progressively. His chest X-ray showed spontaneous pneumothorax with greater severity towards the right side. HRCT revealed multiple innumerable well-defined cystic lesions of variable sizes in bilateral lung fields, more prominent in upper lobes. A negative suction Pleur-evac was advised but the patient refused it and eventually pleurodesis using bleomycin was done to prevent pneumothorax in the future.

Pleurodesis can improve the symptoms of 88 percent of patients without causing any significant complications. Although pleurodesis does not have any effect on patients' survival, it has a positive impact on their lives continuously by enhancing the quality of life.