title: Idiopathic desquamative interstitial pneumonia in a child: a case report.
abstract: BACKGROUND
  Desquamative interstitial pneumonia is a rare form of interstitial lung disease in children. Respiratory symptoms appear progressively, are often subtle, and diagnosis is often delayed by a mean of 6 months after onset. High resolution chest computed tomography is the most sensitive imaging technique for demonstrating and identifying interstitial pneumonia. The typical histologic pattern of desquamative interstitial pneumonia, with prominent clustered alveolar macrophages, diffuse reactive alveolar epithelial hyperplasia and globular proteinaceous material, is diagnostic. Desquamative interstitial pneumonia in children can be idiopathic, though it is mostly related to an inborn error of surfactant metabolism.
  
  
  METHODS
  We present the complex clinical course and pathologic findings of a 30-months-old Mauritian and Senegalese girl with idiopathic desquamative interstitial pneumonia and multiple extrapulmonary manifestations. To our knowledge, this is the first case report of desquamative interstitial pneumonia to occur as part of a syndrome with multiple organ involvement.
  
  
  CONCLUSIONS
  We believe that desquamative interstitial pneumonia is not always associated with mutations of the surfactant proteins, and can still be idiopathic, especially when occurring as part of a syndrome with multiple organ involvement, as described in other interstitial lung diseases.
authors: Pediatric Pulmonology Unit, Department of Pediatrics, Centre Hospitalier Universitaire Vaudois (CHUV), Rue du Bugnon 46, 1011 Lausanne, Switzerland. sabrina.bressieux-degueldre@chuv.ch.
authors: Sabrina
authors: S
authors: Bressieux-Degueldre
authors: Samuel
authors: S
authors: Rotman
authors: Gaudenz
authors: G
authors: Hafen
authors: John-David
authors: JD
authors: Aubert
authors: Isabelle
authors: I
authors: Rochat
mesh_terms: D002675:Child, Preschool; D002658:Developmental Disabilities / Q000150:complications; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000150:complications; D008168:Lung / Q000473:pathology*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000209:etiology; D012871:Skin Diseases / Q000150:complications