The Turkish Journal of Pediatrics 2010 , Vol 52 , Num 6
Langerhans Cell Histiocytosis with Pulmonary Involvement and Unilateral Pneumothorax
Departments of Pediatric Respiratory Diseases and 2Radiology, Mofid Children's Hospital, Shahid Beheshti Medical University, and 3Research Group for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, and ^Molecular Immunology Research Center and Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran Khanbabaee G, Hassas Yeganeh M, Tabatabaei SA, Khatami A, Bazrafshan S, Rezaei N. Langerhans cell histiocytosis with pulmonary involvement and unilateral pneumothorax. Turk J Pediatr 2010; 52: 638-641.

Langerhans cell histiocytosis (LCH) is a rare disorder of Langerhans cell with unknown etiology, which can uncommonly be associated with pneumothorax.

A 14-month-old female is presented here who was referred to our center due to acute respiratory distress. Reticulonodular changes with multiple cystic areas were detected in chest X-ray, whilst extensive honeycombing and cystic changes were seen in high-resolution computed tomography scan. With deterioration of respiratory distress, chest X-ray was repeated, which revealed a unilateral pneumothorax. Meanwhile, some hyperpigmented skin plaques appeared on her skull and back. The biopsy results confirmed the diagnosis of proliferative histiocytosis. Prompt diagnosis of LCH and initiation of appropriate treatment in the patients who present with pneumothorax are vital to prevent further complications and even death in this group of patients. Keywords : histiocytic infiltration, Langerhans cell histiocytosis, pneumothorax

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