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Langerhans cell histiocytosis with pulmonary involvement and unilateral pneumothorax
Ghamartaj Khanbabaee1, Mehrnoosh Hassas Yeganeh, Seyed Ahmad Tabatabaei
1Department of Pediatric Respiratory Diseases, Mofid Children's Hospital, Shahid Beheshti Medical University, Iran.
Insights
Langerhans cell histiocytosis (LCH), a rare condition, can manifest with pneumothorax in infants. Early diagnosis and treatment are crucial for managing this rare lung disease in children.
Area of Science:
- Pediatric Pulmonology
- Dermatology
- Histiocytosis Research
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of Langerhans cells with an unknown cause.
- While LCH commonly affects children, its association with pulmonary complications like pneumothorax is infrequent.
- Understanding the diverse presentations of LCH is vital for timely diagnosis and management.
Observation:
- A 14-month-old female infant presented with acute respiratory distress and skin lesions.
- Radiographic findings included reticulonodular changes, cystic lung disease, and subsequently, a unilateral pneumothorax.
- Hyperpigmented skin plaques on the skull and back were noted, alongside respiratory symptoms.
Findings:
- Chest imaging revealed extensive cystic changes and honeycombing, indicative of severe lung involvement.
- The development of pneumothorax highlighted a critical respiratory complication in this pediatric case.
- Histopathological analysis of a biopsy confirmed proliferative histiocytosis, consistent with LCH.
Implications:
- This case underscores the importance of considering LCH in pediatric patients presenting with unexplained respiratory distress and pneumothorax.
- Prompt diagnosis of LCH is critical to prevent life-threatening complications and improve patient outcomes.
- Integrated management involving pulmonology and dermatology is essential for comprehensive care of LCH patients.
Abstract:
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.
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