title: Langerhans cell histiocytosis presenting with complicated pneumonia, a case report.
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.
authors.affiliation: Department of Paediatrics, Medisch Spectrum Twente, PO Box 50000, 7500 KA Enschede, The Netherlands.
authors.forename: Mark
authors.initials: M
authors.lastname: Ottink
authors.affiliation: Department of Paediatrics, Orbis Medical Centre, PO Box 5500, 6130 MB Sittard, The Netherlands.
authors.forename: Simone
authors.initials: S
authors.lastname: Feijen
authors.affiliation: Department of Paediatrics, Orbis Medical Centre, PO Box 5500, 6130 MB Sittard, The Netherlands.
authors.forename: Philippe
authors.initials: P
authors.lastname: Rosias
authors.affiliation: Department of Radiology, Maastricht University Medical Centre, PO Box 5800, 6202 AZ Maastricht, The Netherlands.
authors.forename: Simon
authors.initials: S
authors.lastname: Robben
authors.affiliation: Paediatric Oncology, Department of Paediatrics, Maastricht University Medical Centre, PO Box 5800, 6202 AZ Maastricht, The Netherlands.
authors.forename: Bernd
authors.initials: B
authors.lastname: Granzen
authors.affiliation: Department of Paediatrics, Orbis Medical Centre, PO Box 5500, 6130 MB Sittard, The Netherlands.
authors.forename: Jan
authors.initials: J
authors.lastname: Heynens
authors.affiliation: Paediatric Pulmonology, Department of Paediatrics, Maastricht University Medical Centre, PO Box 5800, 6202 AZ Maastricht, The Netherlands.
authors.forename: Rijn
authors.initials: R
authors.lastname: Jöbsis
keywords: Children; Cystic lung disease; HRCT, High resolution computed tomography; LCH, Langerhans cell histiocytosis; Langerhans cell histiocytosis; Nail abnormalities; Pleural empyema