Case of disseminated Langerhans' cell histiocytosis presenting with sclerosing cholangitis


DOGANCI T., SAYLI T., Gulderen F., ERDEN E., SENCER H.

INTERNATIONAL JOURNAL OF DERMATOLOGY, vol.43, no.9, pp.673-675, 2004 (SCI-Expanded) identifier identifier identifier

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.