Case of disseminated Langerhans’ cell histiocytosis presenting with sclerosing cholangitis

Authors

  • Tumay Doganci MD,

    Corresponding author
    1. From the Department of Pediatric Gastroenterology, Department of Pediatrics, and Department of Pediatrics, Social Security Ankara Children's Training Hospital, and the Department of Pathology, Medical School of Ankara University, Ankara, Turkey
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  • Tulin Sayli MD,

    1. From the Department of Pediatric Gastroenterology, Department of Pediatrics, and Department of Pediatrics, Social Security Ankara Children's Training Hospital, and the Department of Pathology, Medical School of Ankara University, Ankara, Turkey
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  • Ferda Gulderen MD,

    1. From the Department of Pediatric Gastroenterology, Department of Pediatrics, and Department of Pediatrics, Social Security Ankara Children's Training Hospital, and the Department of Pathology, Medical School of Ankara University, Ankara, Turkey
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  • Esra Erden MD,

    1. From the Department of Pediatric Gastroenterology, Department of Pediatrics, and Department of Pediatrics, Social Security Ankara Children's Training Hospital, and the Department of Pathology, Medical School of Ankara University, Ankara, Turkey
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  • Huriye Sencer MD

    1. From the Department of Pediatric Gastroenterology, Department of Pediatrics, and Department of Pediatrics, Social Security Ankara Children's Training Hospital, and the Department of Pathology, Medical School of Ankara University, Ankara, Turkey
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Tümay Doganci SSK Ankara Çocuk Eḡitim Hastanesi Diþkapi Ankara Turkey E-mail: levdog@gata.edu.tr

Abstract

Langerhans’ cell histiocytosis is an uncommon disorder of childhood with a wide clinical spectrum. Although liver involvement is common in the disseminated form, presentation with hepatic disfunction is unusual. We describe an 18-month-old girl who presented with intrahepatic cholestasis. The patient was shown to have Langerhans’ cell histiocytosis with sclerosing cholangitis by liver biopsy and skin biopsy showing S-100, CD1a positivity, and Birbeck granules by electron microscopy.

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