Primary Pulmonary Langerhans Cell Histiocytosis: A Case Report

Abstract

          Pulmonary Langerhans cell histiocytosis (PLCH) is the clonal proliferation of Langerhans cell confined within the lung as an uncommon interstitial lung disease. The patients with PLCH are always asymptomatic while the spontaneous pneumothorax is its third common presentation.  It has rarely been reported in Thai patients. Herein we report an additional case of PLCH. The patient is a 30-year old Thai smoker who presents with sudden onset of left spontaneous pneumothorax without constitutional symptom. He is immediately treated with the intercostal drainage (ICD) and later with thoracotomy and lung repair because of the continuous leakage of the air from the lung. The HRCT reveals left pneumothorax, multifocal cystic lesions in bilateral upper lungs and superior segment of lower lungs, suggesting Langerhans cell histiocytosis. The microscopic pathology of the lung biopsy shows positive staining for S100 and CD1a, the typical findings of PLCH. He is finally diagnosed as PLCH. After lung repair, no pleurodesis, no corticosteroid, no systemic chemotherapy is offered to him. With only smoking cessation, he does not have any recurrent spontaneous pneumothorax within three months of follow-up although the recurrence of ipsilateral pneumothorax is common in case of PLCH who are treated with only ICD without pleurodesis.