title: Disseminated langerhans cell histiocytosis presenting as cholestatic jaundice.
abstract: Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely described. Cholestasis may result from lymph nodes obstructing the porta hepatis. In this report, we describe a case of type II histiocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant lymphadenopathy at the porta.
authors: Medical Officer, Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital , Krishna Nagar, New Delhi, India .
authors: Rohit
authors: R
authors: Kapoor
authors: Assistant Professor, Department of Pediatrics, Albert Einstein College of Medicine, Attending Physician, Division of Pediatric Gastroenterology and Nutrition, Children's Hospital at Montefiore, , 3415 Bainbridge Avenue, Bronx, NY 10467-2490 .
authors: Anthony M
authors: AM
authors: Loizides
authors: Graduate Student, Vardhman Mahavir Medical College and Safdarjung Hospital , New Delhi, India .
authors: Soumya
authors: S
authors: Sachdeva
authors: Ex Professor, Department of Pediatrics, Vardhman Mahavir Medical College, Safdarjung Hospital , New Delhi, India .
authors: Premila
authors: P
authors: Paul
keywords: Cholestasis; Lymphadenopathy; Porta hepatis