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Langerhans cell histiocytosis presenting with complicated pneumonia, a case report
Mark Ottink1, Simone Feijen2, Philippe Rosias2
1Department of Paediatrics, Medisch Spectrum Twente, PO Box 50000, 7500 KA Enschede, The Netherlands.
Abstract:
We describe a 2 ½ year old boy presenting with fever, abdominal pain and splinter haemorrhages of the nails. On further examination there were signs of pneumonia with pleural effusion. This was treated with mini-thoracotomy, drainage and intravenous antibiotics. Further diagnostic workup for underlying causes showed diffuse cystic lung disease, suggestive of Langerhans cell histiocytosis. This was confirmed on pathology specimens, which showed Langerhans cells in lung tissue, nail bed and skin biopsy samples, indicating multisystem Langerhans cell histiocytosis. The patient was treated with Prednisone and Vinblastin according to the LCH-III guidelines. In this case report we give a brief description on cystic lung disease in children, Langerhans cell histiocytosis and associated nail abnormalities.
Insights
This case report highlights a rare pediatric multisystem Langerhans cell histiocytosis case, presenting with fever, abdominal pain, and nail abnormalities alongside cystic lung disease. Early diagnosis and treatment with chemotherapy are crucial for managing this condition.
Area of Science:
- Pediatric Pulmonology
- Pediatric Pathology
- Pediatric Oncology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder of Langerhans cells.
- Multisystem LCH can affect various organs, including lungs, skin, and bone, presenting with diverse clinical manifestations.
- Cystic lung disease in children is uncommon and requires thorough investigation for underlying causes.
Purpose of the Study:
- To report a rare case of multisystem Langerhans cell histiocytosis in a pediatric patient.
- To emphasize the association between cystic lung disease, nail abnormalities, and LCH.
- To discuss diagnostic and therapeutic approaches for pediatric LCH.
Main Methods:
- Case presentation of a 2.5-year-old boy with fever, abdominal pain, and splinter hemorrhages.
- Diagnostic workup including imaging (revealing cystic lung disease), pathology (confirming Langerhans cells), and biopsy samples (lung, nail bed, skin).
- Treatment administered according to LCH-III guidelines using Prednisone and Vinblastin.
Main Results:
- The patient presented with symptoms suggestive of infection, progressing to pneumonia with pleural effusion.
- Histopathological examination confirmed multisystem Langerhans cell histiocytosis involving the lungs, nail bed, and skin.
- Successful treatment was initiated following established LCH-III protocols.
Conclusions:
- Multisystem Langerhans cell histiocytosis should be considered in pediatric patients with unexplained cystic lung disease and systemic symptoms.
- Nail abnormalities, such as splinter hemorrhages, can be an early indicator of LCH.
- Adherence to LCH-III guidelines ensures appropriate and effective management of pediatric LCH.
