Case of disseminated Langerhans' cell histiocytosis presenting with sclerosing cholangitis
INTERNATIONAL JOURNAL OF DERMATOLOGY, vol.43, no.9, pp.673-675, 2004 (SCI-Expanded, Scopus)
- Publication Type: Article / Article
- Volume: 43 Issue: 9
- Publication Date: 2004
- Doi Number: 10.1111/j.1365-4632.2004.02160.x
- Journal Name: INTERNATIONAL JOURNAL OF DERMATOLOGY
- Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Page Numbers: pp.673-675
- Ankara University Affiliated: No
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.